#METABOLOMICS WORKBENCH Jung_DNLI_20250623_151403 DATATRACK_ID:6083 STUDY_ID:ST004058 ANALYSIS_ID:AN006707 PROJECT_ID:PR002548 VERSION 1 CREATED_ON July 22, 2025, 11:13 am #PROJECT PR:PROJECT_TITLE Lysosomal polyamine storage upon ATP13A2 loss impairs β-glucocerebrosidase via PR:PROJECT_TITLE altered lysosomal pH and electrostatic hydrolase-lipid interactions PR:PROJECT_TYPE Preclinical Mouse and cellular studies PR:PROJECT_SUMMARY ATP13A2 is an endolysosomal polyamine transporter mutated in several PR:PROJECT_SUMMARY neurodegenerative conditions involving lysosomal defects, including PR:PROJECT_SUMMARY Parkinson’s disease (PD). While polyamines are polybasic and polycationic PR:PROJECT_SUMMARY molecules that play pleiotropic cellular roles, their specific impact on PR:PROJECT_SUMMARY lysosomal health is unknown. Here, we demonstrate lysosomal polyamine PR:PROJECT_SUMMARY accumulation in ATP13A2 knockout (KO) cell lines. Primary polyamine storage PR:PROJECT_SUMMARY caused secondary storage of lysosomal anionic phospholipid PR:PROJECT_SUMMARY bis(monoacylglycero)phosphate (BMP) and age-dependent increase in the PR:PROJECT_SUMMARY β-glucocerebrosidase (GCase) substrate, glucosylsphingosine, in Atp13a2 KO PR:PROJECT_SUMMARY brains. Polyamine accumulation inhibited lysosomal GCase activity in cells and PR:PROJECT_SUMMARY this was reversed by lysosome reacidification or BMP supplementation. A PR:PROJECT_SUMMARY liposome-based GCase assay utilizing physiological substrates demonstrated PR:PROJECT_SUMMARY dose-dependent inhibition of BMP-stimulated GCase activity by polyamines, in PR:PROJECT_SUMMARY part via a pH-independent, electrostatics-based mechanism. Therefore, excess PR:PROJECT_SUMMARY polyamine compromises lysosomes by disrupting pH and electrostatic interactions PR:PROJECT_SUMMARY between GCase and BMP enabling efficient substrate hydrolysis, potentially PR:PROJECT_SUMMARY clarifying their pathogenic mechanisms, and suggesting convergence on PR:PROJECT_SUMMARY PD-relevant pathways. PR:INSTITUTE Denali Therapeutics PR:LAST_NAME Suh PR:FIRST_NAME Jung PR:ADDRESS 161 Oyster Point Blvd, South San Francisco, California, 94080, USA PR:EMAIL suh@dnli.com PR:PHONE +1 6507973837 #STUDY ST:STUDY_TITLE Targeted Lipid and Metabolite Profiling in ATP13A2 knockout (KO) in HAP1 cells ST:STUDY_SUMMARY Targeted profiling of lipids and metabolites was performed in ATP13A2 knockout ST:STUDY_SUMMARY (KO) HAP1 cells with and without polyamine (Spermine) and lipid (DOPC or PG ST:STUDY_SUMMARY 22:6) treatments. ST:INSTITUTE Denali Therapeutics ST:LAST_NAME Suh ST:FIRST_NAME Jung ST:ADDRESS 161 Oyster Point Blvd ST:EMAIL suh@dnli.com ST:PHONE +1 6507973837 #SUBJECT SU:SUBJECT_TYPE Cultured cells SU:SUBJECT_SPECIES Homo sapiens SU:TAXONOMY_ID 9606 #SUBJECT_SAMPLE_FACTORS: SUBJECT(optional)[tab]SAMPLE[tab]FACTORS(NAME:VALUE pairs separated by |)[tab]Raw file names and additional sample data SUBJECT_SAMPLE_FACTORS Pool_001 HSA-000043722 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_002.mzML SUBJECT_SAMPLE_FACTORS KO_b HSA-000043633 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_003.mzML SUBJECT_SAMPLE_FACTORS WT_b HSA-000043625 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_007.mzML SUBJECT_SAMPLE_FACTORS WT_a HSA-000043624 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_009.mzML SUBJECT_SAMPLE_FACTORS WT_c HSA-000043626 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_010.mzML SUBJECT_SAMPLE_FACTORS Pool_002 HSA-000043812 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_011.mzML SUBJECT_SAMPLE_FACTORS WT_d HSA-000043627 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_014.mzML SUBJECT_SAMPLE_FACTORS KO_a HSA-000043632 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_015.mzML SUBJECT_SAMPLE_FACTORS KO_c HSA-000043634 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_016.mzML SUBJECT_SAMPLE_FACTORS KO_d HSA-000043635 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_018.mzML SUBJECT_SAMPLE_FACTORS Pool_003 HSA-000043813 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000956_HALO_Pos_020.mzML SUBJECT_SAMPLE_FACTORS Pool1_001 HSA-000043814 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_002.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_002.mzML SUBJECT_SAMPLE_FACTORS pH_KO_c HSA-000043739 Genotype:ATP13A2 KO | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_003.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_003.mzML SUBJECT_SAMPLE_FACTORS pH_WT_b HSA-000043726 Genotype:WT | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_004.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_004.mzML SUBJECT_SAMPLE_FACTORS pH_WT_SPM_b HSA-000043729 Genotype:WT | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_005.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_005.mzML SUBJECT_SAMPLE_FACTORS pH_KO_Rim_a HSA-000043743 Genotype:ATP13A2 KO | Treatment:Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_006.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_006.mzML SUBJECT_SAMPLE_FACTORS pH_WT_SPM_Rim_c HSA-000043736 Genotype:WT | Treatment:SPM + Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_007.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_007.mzML SUBJECT_SAMPLE_FACTORS pH_KO_SPM_Rim_b HSA-000043747 Genotype:ATP13A2 KO | Treatment:SPM + Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_008.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_008.mzML SUBJECT_SAMPLE_FACTORS pH_WT_Rim_a HSA-000043731 Genotype:WT | Treatment:Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_009.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_009.mzML SUBJECT_SAMPLE_FACTORS pH_WT_Rim_b HSA-000043732 Genotype:WT | Treatment:Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_010.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_010.mzML SUBJECT_SAMPLE_FACTORS pH_WT_SPM_Rim_b HSA-000043735 Genotype:WT | Treatment:SPM + Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_011.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_011.mzML SUBJECT_SAMPLE_FACTORS pH_KO_SPM_c HSA-000043742 Genotype:ATP13A2 KO | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_012.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_012.mzML SUBJECT_SAMPLE_FACTORS pH_KO_SPM_Rim_a HSA-000043746 Genotype:ATP13A2 KO | Treatment:SPM + Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_013.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_013.mzML SUBJECT_SAMPLE_FACTORS pH_KO_a HSA-000043737 Genotype:ATP13A2 KO | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_014.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_014.mzML SUBJECT_SAMPLE_FACTORS Pool1_002 HSA-000043815 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_015.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_015.mzML SUBJECT_SAMPLE_FACTORS pH_WT_Rim_c HSA-000043733 Genotype:WT | Treatment:Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_016.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_016.mzML SUBJECT_SAMPLE_FACTORS pH_KO_SPM_a HSA-000043740 Genotype:ATP13A2 KO | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_017.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_017.mzML SUBJECT_SAMPLE_FACTORS pH_WT_SPM_a HSA-000043728 Genotype:WT | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_018.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_018.mzML SUBJECT_SAMPLE_FACTORS pH_WT_SPM_c HSA-000043730 Genotype:WT | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_019.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_019.mzML SUBJECT_SAMPLE_FACTORS pH_KO_SPM_Rim_c HSA-000043748 Genotype:ATP13A2 KO | Treatment:SPM + Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_020.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_020.mzML SUBJECT_SAMPLE_FACTORS pH_KO_Rim_b HSA-000043744 Genotype:ATP13A2 KO | Treatment:Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_021.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_021.mzML SUBJECT_SAMPLE_FACTORS pH_WT_a HSA-000043725 Genotype:WT | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_022.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_022.mzML SUBJECT_SAMPLE_FACTORS pH_WT_c HSA-000043727 Genotype:WT | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_023.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_023.mzML SUBJECT_SAMPLE_FACTORS pH_WT_SPM_Rim_a HSA-000043734 Genotype:WT | Treatment:SPM + Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_024.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_024.mzML SUBJECT_SAMPLE_FACTORS pH_KO_SPM_b HSA-000043741 Genotype:ATP13A2 KO | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_025.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_025.mzML SUBJECT_SAMPLE_FACTORS pH_KO_Rim_c HSA-000043745 Genotype:ATP13A2 KO | Treatment:Rim | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_026.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_026.mzML SUBJECT_SAMPLE_FACTORS pH_KO_b HSA-000043738 Genotype:ATP13A2 KO | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_027.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_027.mzML SUBJECT_SAMPLE_FACTORS Pool1_003 HSA-000043816 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_028.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_028.mzML SUBJECT_SAMPLE_FACTORS Pool2_001 HSA-000043817 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_030.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_030.mzML SUBJECT_SAMPLE_FACTORS PG_WT_c HSA-000043754 Genotype:WT | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_031.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_031.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_DOPC_b HSA-000043759 Genotype:WT | Treatment:SPM + DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_033.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_033.mzML SUBJECT_SAMPLE_FACTORS PG_KO_22 6 PG_b HSA-000043798 Genotype:ATP13A2 KO | Treatment:22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_034.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_034.mzML SUBJECT_SAMPLE_FACTORS PG_WT_DOPC_c HSA-000043769 Genotype:WT | Treatment:DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_035.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_035.mzML SUBJECT_SAMPLE_FACTORS PG_KO_c HSA-000043778 Genotype:ATP13A2 KO | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_036.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_036.mzML SUBJECT_SAMPLE_FACTORS PG_KO_22 6 PG_a HSA-000043797 Genotype:ATP13A2 KO | Treatment:22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_037.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_037.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_22 6 PG_b HSA-000043789 Genotype:ATP13A2 KO | Treatment:SPM + 22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_038.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_038.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_c HSA-000043757 Genotype:WT | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_039.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_039.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_DOPC_c HSA-000043760 Genotype:WT | Treatment:SPM + DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_040.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_040.mzML SUBJECT_SAMPLE_FACTORS PG_KO_a HSA-000043776 Genotype:ATP13A2 KO | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_041.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_041.mzML SUBJECT_SAMPLE_FACTORS PG_WT_22 6 PG_c HSA-000043775 Genotype:WT | Treatment:22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_042.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_042.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_a HSA-000043755 Genotype:WT | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_043.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_043.mzML SUBJECT_SAMPLE_FACTORS PG_WT_b HSA-000043753 Genotype:WT | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_044.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_044.mzML SUBJECT_SAMPLE_FACTORS PG_KO_22 6 PG_c HSA-000043799 Genotype:ATP13A2 KO | Treatment:22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_045.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_045.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_DOPC_a HSA-000043782 Genotype:ATP13A2 KO | Treatment:SPM + DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_046.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_046.mzML SUBJECT_SAMPLE_FACTORS PG_WT_DOPC_b HSA-000043768 Genotype:WT | Treatment:DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_049.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_049.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_22 6 PG_c HSA-000043766 Genotype:WT | Treatment:SPM + 22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_051.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_051.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_DOPC_a HSA-000043758 Genotype:WT | Treatment:SPM + DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_054.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_054.mzML SUBJECT_SAMPLE_FACTORS Pool2_002 HSA-000043818 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_055.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_055.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_22 6 PG_a HSA-000043764 Genotype:WT | Treatment:SPM + 22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_057.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_057.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_22 6 PG_c HSA-000043790 Genotype:ATP13A2 KO | Treatment:SPM + 22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_059.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_059.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_DOPC_c HSA-000043784 Genotype:ATP13A2 KO | Treatment:SPM + DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_060.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_060.mzML SUBJECT_SAMPLE_FACTORS PG_KO_DOPC_c HSA-000043793 Genotype:ATP13A2 KO | Treatment:DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_062.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_062.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_b HSA-000043756 Genotype:WT | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_063.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_063.mzML SUBJECT_SAMPLE_FACTORS PG_WT_22 6 PG_a HSA-000043773 Genotype:WT | Treatment:22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_064.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_064.mzML SUBJECT_SAMPLE_FACTORS PG_KO_DOPC_b HSA-000043792 Genotype:ATP13A2 KO | Treatment:DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_065.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_065.mzML SUBJECT_SAMPLE_FACTORS PG_KO_DOPC_a HSA-000043791 Genotype:ATP13A2 KO | Treatment:DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_066.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_066.mzML SUBJECT_SAMPLE_FACTORS PG_WT_a HSA-000043752 Genotype:WT | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_067.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_067.mzML SUBJECT_SAMPLE_FACTORS PG_KO_b HSA-000043777 Genotype:ATP13A2 KO | Treatment:PBS | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_069.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_069.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_DOPC_b HSA-000043783 Genotype:ATP13A2 KO | Treatment:SPM + DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_070.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_070.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_c HSA-000043781 Genotype:ATP13A2 KO | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_071.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_071.mzML SUBJECT_SAMPLE_FACTORS PG_WT_DOPC_a HSA-000043767 Genotype:WT | Treatment:DOPC | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_072.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_072.mzML SUBJECT_SAMPLE_FACTORS PG_WT_22 6 PG_b HSA-000043774 Genotype:WT | Treatment:22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_075.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_075.mzML SUBJECT_SAMPLE_FACTORS PG_WT_SPM_22 6 PG_b HSA-000043765 Genotype:WT | Treatment:SPM + 22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_076.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_076.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_a HSA-000043779 Genotype:ATP13A2 KO | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_077.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_077.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_b HSA-000043780 Genotype:ATP13A2 KO | Treatment:SPM | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_078.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_078.mzML SUBJECT_SAMPLE_FACTORS PG_KO_SPM_22 6 PG_a HSA-000043788 Genotype:ATP13A2 KO | Treatment:SPM + 22 6 PG | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_079.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_079.mzML SUBJECT_SAMPLE_FACTORS Pool2_003 HSA-000043819 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=-; RAW_FILE_NAME(Raw Source Name.LN)=20250124_MS_HEX-000957_Lipid_Neg_080.mzML; RAW_FILE_NAME(Raw Source Name.MP)=-; RAW_FILE_NAME(Raw Source Name.MN)=-; RAW_FILE_NAME(Raw Source Name.HALO)=20250122_MS_HEX-000957_HALO_Pos_080.mzML SUBJECT_SAMPLE_FACTORS Pool_Lyso_001 HSA-000038939 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_002.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_002.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_002.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS WT #1 Lyso HSA-000038932 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_003.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_003.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_003.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS WT #2 Lyso HSA-000038933 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_007.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_007.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_007.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS Pool_Lyso_002 HSA-000040325 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_008.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_008.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_008.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS WT #3 Lyso HSA-000038934 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_009.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_009.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_009.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS KO #2 Lyso HSA-000038936 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_011.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_011.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_011.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS Pool_Lyso_003 HSA-000040326 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_016.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_016.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_016.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS KO #1 Lyso HSA-000038935 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_019.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_019.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_019.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS KO #3 Lyso HSA-000038937 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_022.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_022.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_022.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS Pool_Lyso_004 HSA-000040327 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_023.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_023.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_023.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS Pool_WCL_001 HSA-000038938 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_024.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_024.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_024.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS KO #1 WCL HSA-000038929 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_028.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_028.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_028.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS KO #2 WCL HSA-000038930 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_029.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_029.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_029.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS KO #3 WCL HSA-000038931 Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_030.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_030.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_030.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS Pool_WCL_002 HSA-000040322 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_031.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_031.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_031.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS WT #2 WCL HSA-000038927 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_036.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_036.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_036.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS WT #1 WCL HSA-000038926 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_037.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_037.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_037.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS Pool_WCL_003 HSA-000040323 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_038.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_038.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_038.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS WT #3 WCL HSA-000038928 Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_043.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_043.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_043.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- SUBJECT_SAMPLE_FACTORS Pool_WCL_004 HSA-000040324 Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate RAW_FILE_NAME(Raw Source Name.BZC)=20240905_MS_HEX-000820_PolyA_BzC_Pos_045.mzML; RAW_FILE_NAME(Raw Source Name.LN)=-; RAW_FILE_NAME(Raw Source Name.MP)=20240904_MS_HEX-000820_Met_Pos_045.mzML; RAW_FILE_NAME(Raw Source Name.MN)=20240905_MS_HEX-000820_Met_Neg_045.mzML; RAW_FILE_NAME(Raw Source Name.HALO)=- #COLLECTION CO:COLLECTION_SUMMARY Human HAP1 parental WT and ATP13A2 KO cells (~25,000) were washed 1x with 0.9% CO:COLLECTION_SUMMARY sodium chloride and harvested for metabolite and lipid extraction. Lysosomal CO:COLLECTION_SUMMARY fractions were isolated from HAP1 parental WT and ATP13A2 KO cells following CO:COLLECTION_SUMMARY endocytosis-mediated labeling using superparamagnetic iron oxide nanoparticles CO:COLLECTION_SUMMARY (SPION). HAP1 cells were seeded in 3X 10cm tissue culture dishes and allowed to CO:COLLECTION_SUMMARY grow till ~75% confluency at which time they were treated for 24 h with 10% CO:COLLECTION_SUMMARY (v/v) dextran-magnetite solution (DexoMAG40, Liquids Research Ltd.). The cells CO:COLLECTION_SUMMARY were rinsed with warmed 1X PBS, pH 7.4 (Gibco; # 10010023) to remove residual CO:COLLECTION_SUMMARY extracellular dextran-magnetite and re-supplemented with fresh culture medium CO:COLLECTION_SUMMARY for at least 4 h to allow for specific labeling of lysosomes and late endosomes. CO:COLLECTION_SUMMARY Cells were harvested by scraping with ice cold 1X PBS and centrifugation at 300 CO:COLLECTION_SUMMARY x g for 5 min at 4°C; resuspended in isolation buffer (250mM sucrose, 10 mM CO:COLLECTION_SUMMARY HEPES, 1 mM CaCl2, 1 mM MgCl2, 1 mM DTT, cOmplete Protease Inhibitor (Roche; CO:COLLECTION_SUMMARY #04693132001)), and replicates pooled by centrifugation into 1 mL lysis buffer. CO:COLLECTION_SUMMARY Cells were lysed using a 27G needle for 20 strokes, followed by centrifugation CO:COLLECTION_SUMMARY of the cell lysate at 600 x g for 10 min at 4°C. Post-nuclear supernatant (PNS) CO:COLLECTION_SUMMARY was collected, and pellet was resuspended in 1 mL isolation buffer followed by CO:COLLECTION_SUMMARY another round of needle lysis and PNS collection to capture maximum lysosomes. CO:COLLECTION_SUMMARY Pooled supernatants from both rounds of needle lysis were then loaded onto LS CO:COLLECTION_SUMMARY columns (Miltenyi Biotec; #130-042-401), attached to Quadro MACS magnet, that CO:COLLECTION_SUMMARY were already pre equilibrated with chilled isolation buffer. Flowthrough was CO:COLLECTION_SUMMARY collected after passing the PNS through the column by gravity flow and passed CO:COLLECTION_SUMMARY through the column again to increase retention of unbound labeled lysosomes. CO:COLLECTION_SUMMARY Finally, the columns were washed three times with isolation buffer to eliminate CO:COLLECTION_SUMMARY other membrane-bound organelle contaminants, and magnetically bound lysosomes CO:COLLECTION_SUMMARY eluted twice into 0.5 mL isolation buffer. The elutes were diluted with 1 mL 1X CO:COLLECTION_SUMMARY PBS, centrifuged at 21,000 x g for 40 min at 4°C. The lysosomal pellet was CO:COLLECTION_SUMMARY resuspended in 200 µL isolation buffer and harvested for metabolite and lipid CO:COLLECTION_SUMMARY extraction. CO:SAMPLE_TYPE HAP1 cells CO:STORAGE_CONDITIONS -80℃ CO:COLLECTION_VIALS Lobind 1.5 mL Eppendorf tubes CO:STORAGE_VIALS Lobind 1.5 mL Eppendorf tubes #TREATMENT TR:TREATMENT_SUMMARY Human HAP1 parental WT and ATP13A2 KO cells (Horizon Discovery; WT: C631; TR:TREATMENT_SUMMARY ATP13A2 KO: HZGHC003547c002) were cultured in Iscove’s Modified Dulbecco’s TR:TREATMENT_SUMMARY Medium (IMDM, Gibco; #12440053) supplemented with 10% fetal bovine serum (VWR TR:TREATMENT_SUMMARY Seradigm; #97068-085) and 1% Penicillin/Streptomycin (Gibco; #15140122). No TR:TREATMENT_SUMMARY treatment.Cells were treated for 20 hours with PBS, Rimeporide(Rim), Spermine TR:TREATMENT_SUMMARY (SPM), or a combination of Spermine (SPM) + Rimeporide(Rim), followed by TR:TREATMENT_SUMMARY metabolite extraction. Cells were treated for 20 hours with PBS , Spermine TR:TREATMENT_SUMMARY (SPM), 18:1 (Δ9-Cis) PC (DOPC), 22:6 PG, a combination of Spermine(SPM) + DOPC, TR:TREATMENT_SUMMARY or a combination of Spermine (SPM) + 22:6 PG,followed by metabolite extraction. TR:TREATMENT_SUMMARY NOTE: To evaluate whether BMP can rescue spermine-dependent inhibition, WT HAP1 TR:TREATMENT_SUMMARY cells were treated with spermine and supplemented with PG(22:6/22:6), a TR:TREATMENT_SUMMARY structural isomer and precursor of BMP(22:6/22:6). PG (22:6/22:6) was TR:TREATMENT_SUMMARY supplemented due to its lower cost and greater availability, relying on its TR:TREATMENT_SUMMARY established rapid conversion to BMP in lysosomes (see TR:TREATMENT_SUMMARY 10.1016/j.bbalip.2021.158916). #SAMPLEPREP SP:SAMPLEPREP_SUMMARY Cells (~25,000) and lysosomal fractions (100 µg) were directly lysed and SP:SAMPLEPREP_SUMMARY extracted in 150 µL methanol containing stable-isotope internal standards for SP:SAMPLEPREP_SUMMARY 20 min at 4°C with shaking. The supernatant fraction was transferred to a SP:SAMPLEPREP_SUMMARY 96-well sample collection plate (Waters; #186005837) following a 14,000 x g spin SP:SAMPLEPREP_SUMMARY at 4°C for 20 min from which the supernatant was distributed for the LC-MS/MS SP:SAMPLEPREP_SUMMARY analyses. SP:PROCESSING_STORAGE_CONDITIONS On ice SP:EXTRACT_STORAGE -20℃ #CHROMATOGRAPHY CH:CHROMATOGRAPHY_TYPE Ion exchange CH:INSTRUMENT_NAME Agilent 1290 Infinity II CH:COLUMN_NAME Imtakt Intrada Organic Acid (150 × 2 mm, 3 um) CH:SOLVENT_A 10% acetonitrile/90% water; 0.1% formic acid CH:SOLVENT_B 10% acetonitrile/90% water; 100mM ammonium formate CH:FLOW_GRADIENT 0.0-1.0 min at 0% B; 1.0-7.0 min to 100% B; 7.1 at 0% B; and 7.1-10 min at 0% B. CH:FLOW_RATE 0.20 mL/min CH:COLUMN_TEMPERATURE 60 CH:SAMPLE_INJECTION 5 #ANALYSIS AN:ANALYSIS_TYPE MS #MS MS:INSTRUMENT_NAME ABI Sciex 6500+ QTrap MS:INSTRUMENT_TYPE Triple quadrupole MS:MS_TYPE ESI MS:ION_MODE NEGATIVE MS:MS_COMMENTS curtain gas at 30 psi; collision gas was set at medium; ion spray voltage at MS:MS_COMMENTS -4,500 V; temperature at 600°C; ion source Gas 1 at 50 psi; ion source Gas 2 at MS:MS_COMMENTS 60 psi #MS_METABOLITE_DATA MS_METABOLITE_DATA:UNITS normalized peak area MS_METABOLITE_DATA_START Samples HSA-000038939 HSA-000038932 HSA-000038933 HSA-000040325 HSA-000038934 HSA-000038936 HSA-000040326 HSA-000038935 HSA-000038937 HSA-000040327 HSA-000038938 HSA-000038929 HSA-000038930 HSA-000038931 HSA-000040322 HSA-000038927 HSA-000038926 HSA-000040323 HSA-000038928 HSA-000040324 Factors Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:lysosome Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:lysosome Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:lysosome Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:lysosome Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:lysosome Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:lysosome Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:lysosome Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:ATP13A2 KO | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:WT | Treatment:none | Sample source:HAP1 | Sample Type:whole cell lysate Genotype:NA | Treatment:NA | Sample source:HAP1 | Sample Type:whole cell lysate 13C3-Citric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 13C4-Fumaric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 13C4-Malic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 13C4-Succinic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 13C5-Itaconic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 13C5-Oxoglutaric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 15N5-ADP N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 2-Hydroxyglutarate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 2-Phosphoglyceric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 3-Hydroxybutyric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 3-Phosphoglyceric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 6-Phosphogluconic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. ADP N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. 1.779 7.14 3.536 26.44 3.377 30.925 69.12 3.962 56.329 4.217 Acetoacetic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Adenosine monophosphate 0.093 0.006 0.03 0.094 0.035 0.75 0.105 0.006 0.35 0.1 1.959 75.78 10.127 48.59 1.894 55.969 106.618 1.973 68.753 1.883 Adenosine triphosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Adenosine triphosphate-d4 N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Alanine N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Alanine-d4 N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Arginine N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Arginine-d4C13 N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Asparagine N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Aspartic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Aspartic acid-d3 N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Dihydroxyacetone phosphate 0.22 0.119 0.071 0.553 0.075 0.747 0.308 0.124 1.15 0.455 61.764 873.38 93.22 2209.636 62.338 886.991 1563.334 55.938 1374.108 58.052 Erythose 4-phosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Fructose 1,6-bisphosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Fructose 1-phosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Fructose 6-phosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Fumaric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Glucose 1-phosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Glucose 6-phosphate 3.619 5.715 3.55 3.928 5.09 13.127 3.838 3.173 11.898 3.826 45.563 345.098 305.388 563.029 43.533 411.679 449.954 45.701 399.789 45.261 Glutamic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Glutamic acid-d3 N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Glutamine N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Glyceraldehyde 3-phosphate 0.688 0.156 0.271 0.279 0.201 0.578 0.638 0.094 1.479 0.657 82.927 1160.502 116.723 2895.919 85.038 1161.41 2063.026 74.168 1809.27 75.576 Itaconic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Lactic acid 4.9 5.454 2.685 5.029 3.639 7.034 4.94 4.948 4.552 4.649 29.495 170.59 248.929 323.79 27.884 143.01 188.927 26.454 265.511 28.066 Malic acid 0.933 1.564 0.79 0.767 0.982 1.776 0.729 0.684 1.704 0.681 20.37 594.055 583.58 511.734 18.273 456.212 653.822 19.608 575.571 19.6 NAD N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. NADH N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. NADP N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. NADPH N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Oxalacetic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Oxoglutaric acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Phosphocreatine N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Phosphoenolpyruvic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Pyruvic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Ribose 5-phosphate 0.193 0.597 0.128 0.18 0.851 3.853 0.161 0.142 3.594 0.161 25.923 259.558 287.061 316.553 26.42 170.818 354.643 25.42 206.452 26.237 Ribulose 5-phosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Sedoheptulose 7-phosphate 0.447 0.292 0.266 0.415 0.355 2.351 0.44 0.141 0.93 0.459 8.862 141.955 147.634 200.164 8.487 82.558 311.76 7.796 141.665 8.677 Serine N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Succinic acid 7.438 10.499 11.985 8.387 11.819 13.489 7.976 7.27 9.983 7.057 14.979 156.882 285.391 118.081 14.531 155.753 265.752 14.888 168.34 14.901 Sum of Citric acid and Isocitric acid 20.575 33.082 16.986 14.489 19.688 13.915 10.198 10.413 14.63 6.954 13.641 155.221 190.452 169.771 22.322 151.018 242.748 27.33 195.701 29.56 U-13C10,U-15N5-Adenosine monophosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. U-13C3-Pyruvic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. U-13C6-Glucose 6-phosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. UDP-Glucose N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. Xylulose 5-phosphate N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. cis-Aconitic acid N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. N.D. MS_METABOLITE_DATA_END #METABOLITES METABOLITES_START metabolite_name Expected RT MS1_mz MS2_mz 13C3-Citric acid 4.61 194.2 113 13C4-Fumaric acid 4.48 119.2 74.3 13C5-Itaconic acid 2.67 134 89.2 13C4-Malic acid 3.37 137.2 119.2 13C4-Succinic acid 2.32 121.3 76 13C5-Oxoglutaric acid 4.7 150.2 105.3 15N5-ADP 5.95 431.1 79 2-Hydroxyglutarate 2.9 145 101 2-Phosphoglyceric acid 5.66 185.5 97.1 3-Hydroxybutyric acid 2.17 103.2 59.2 3-Phosphoglyceric acid 5.67 185.5 97 6-Phosphogluconic acid 5.3 275 97 Acetoacetic acid 0.31 101 101 Adenosine monophosphate 3.9 346 79 Adenosine triphosphate 7 506.1 158.9 Adenosine triphosphate-d4 7 510 158.9 ADP 5.97 426.1 79 Alanine 2.18 88 88 Alanine-d4 2.07 92 92 Arginine 1.24 173 131 Arginine-d4C13 1.24 178 136 Asparagine 1.78 131 114 Aspartic acid 2.25 132 88 Aspartic acid-d3 2.25 135 91 cis-Aconitic acid 5.33 173 129 Sum of Citric acid and Isocitric acid 4.54 191.2 111 Dihydroxyacetone phosphate 4.55 169.1 79 Erythose 4-phosphate 4.36 199 97 Fructose 1,6-bisphosphate 5.51 339.1 79 Fructose 6-phosphate 4.5 259 97.1 Fumaric acid 4.7 115.2 71.3 Glucose 1-phosphate 4.5 259 241 Glucose 6-phosphate 4.5 259 97 Glutamic acid 1.9 146 128 Glutamic acid-d3 1.91 149 131 Glutamine 1.78 145 127 Glyceraldehyde 3-phosphate 4.55 169.1 97 Itaconic acid 2.67 129.2 85 Lactic acid 2.5 89.2 43.2 Malic acid 3.36 133.2 115.2 NAD 3.77 662.1 540.3 NADH 2.87 664.1 408.1 NADP 5.97 742.1 620 NADPH 4.73 744.1 408 Oxalacetic acid 3.63 131 131 Oxoglutaric acid 4.87 145.2 101.3 Phosphocreatine 3.53 210 79 Phosphoenolpyruvic acid 5.78 167 79 Pyruvic acid 4.64 87.2 43.2 Ribose 5-phosphate 4.54 229 97 Ribulose 5-phosphate 4.42 229 97.1 Sedoheptulose 7-phosphate 4.41 289 97 Serine 1.77 104 74 Succinic acid 2.28 117.2 73.2 U-13C10,U-15N5-Adenosine monophosphate 3.9 361 79 U-13C3-Pyruvic acid 4.64 90.2 45.2 U-13C6-Glucose 6-phosphate 4.4 265 97 Xylulose 5-phosphate 4.5 229.1 97 Fructose 1-phosphate 4.5 259.1 79 UDP-Glucose 5.19 565.2 323.4 METABOLITES_END #END