ST003347 ADSL deficiency drives mitochondrial dysfunction and ERK2 dysregulation in a linear genotype to phenotype correlation
Limit by pathway class:
   Minimum # of metabolites per pathway:

Pathway name (# of metabolites detected in this study)HMDB pathwayKEGG pathway
Alanine Metabolism (8) SMP0000055 map00250
Amino Sugar Metabolism (9) SMP0000045 map00520
Ammonia Recycling (13) SMP0000009 map00910
Androgen and Estrogen Metabolism (3) SMP0000068 map00150
Androstenedione Metabolism (3) SMP0030406 -
Arachidonic Acid Metabolism (5) SMP0000075 map00590
Arginine and Proline Metabolism (21) SMP0000020 map00330
Aspartate Metabolism (16) SMP0000067 map00250
beta-Alanine Metabolism (10) SMP0000007 map00410
Beta-Alanine Metabolism (1) SMP0000007 map00410
Betaine Metabolism (8) SMP0000123 map00260
Beta Oxidation of Very Long Chain Fatty Acids (3) SMP0000052 map01040
Bile Acid Biosynthesis (7) SMP0000035 map00120
Biotin Metabolism (2) SMP0000066 map00780
Butyrate Metabolism (4) SMP0000073 map00650
Caffeine Metabolism (1) SMP0000028 map00232
Cardiolipin Biosynthesis (3) SMP0020986 -
Carnitine Synthesis (8) SMP0000465 -
Catecholamine Biosynthesis (4) SMP0000012 map00350
Citric Acid Cycle (12) SMP0000057 map00020
Cysteine Metabolism (7) SMP0000013 map00270
Degradation of Superoxides (1) SMP0000468 -
De Novo Triacylglycerol Biosynthesis (3) SMP0015896 -
Estrone Metabolism (5) SMP0030880 -
Ethanol Degradation (5) SMP0000449 -
Fatty Acid Elongation in Mitochondria (3) SMP0000054 map00062
Fatty Acid Metabolism (3) SMP0000051 map00071
Folate Metabolism (6) SMP0000053 map00670
Fructose and Mannose Degradation (10) SMP0000064 map00051
Galactose Metabolism (8) SMP0000043 map00052
Gluconeogenesis (14) SMP0000128 map00010
Glucose-Alanine Cycle (6) SMP0000127 -
Glutamate Metabolism (20) SMP0000072 map00250
Glutathione Metabolism (11) SMP0000015 map00480
Glycerolipid Metabolism (7) SMP0000039 map00561
Glycerol Phosphate Shuttle (4) SMP0000124 -
Glycine and Serine Metabolism (19) SMP0000004 map00260
Glycolysis (9) SMP0000040 map00010
Histidine Metabolism (12) SMP0000044 map00340
Homocysteine Degradation (2) SMP0000455 -
Inositol Metabolism (6) SMP0000011 map00562
Inositol Phosphate Metabolism (5) SMP0000462 map00562
Ketone Body Metabolism (2) SMP0000071 map00072
Lactose Degradation (3) SMP0000457 -
Lactose Synthesis (6) SMP0000444 -
Lysine Degradation (6) SMP0000037 map00310
Malate-Aspartate Shuttle (5) SMP0000129 -
Methionine Metabolism (14) SMP0000033 map00270
Methylhistidine Metabolism (3) SMP0000715 -
Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids (3) SMP0000482 -
Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids (3) SMP0000481 -
Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids (3) SMP0000480 -
Mitochondrial Electron Transport Chain (8) SMP0000355 map00190
Nicotinate and Nicotinamide Metabolism (12) SMP0000048 map00760
Nucleotide Sugars Metabolism (7) SMP0000010 map00520
Oxidation of Branched Chain Fatty Acids (6) SMP0000030 -
Oxidation of Branched-Chain Fatty Acids (6) SMP0000030 -
Pantothenate and CoA Biosynthesis (4) SMP0000027 map00770
Pentose Phosphate Pathway (10) SMP0000031 map00030
Phenylacetate Metabolism (3) SMP0000126 map00360
Phenylalanine and Tyrosine Metabolism (7) SMP0000008 map00360
Phosphatidylcholine Biosynthesis (5) SMP0014212 -
Phosphatidylethanolamine Biosynthesis (4) SMP0029731 -
Phosphatidylinositol Phosphate Metabolism (3) SMP0000463 map00562
Phospholipid Biosynthesis (7) SMP0000025 map00564
Phytanic Acid Peroxisomal Oxidation (5) SMP0000450 -
Plasmalogen Synthesis (1) SMP0000479 -
Porphyrin Metabolism (2) SMP0000024 map00860
Propanoate Metabolism (9) SMP0000016 map00640
Pterine Biosynthesis (4) SMP0000005 map00790
Purine Metabolism (24) SMP0000050 map00230
Pyrimidine Metabolism (13) SMP0000046 map00240
Pyruvaldehyde Degradation (2) SMP0000459 -
Pyruvate Metabolism (12) SMP0000060 map00620
Retinol Metabolism (3) SMP0000074 map00830
Riboflavin Metabolism (3) SMP0000070 map00740
Selenoamino Acid Metabolism (7) SMP0000029 map00450
Spermidine and Spermine Biosynthesis (4) SMP0000445 -
Sphingolipid Metabolism (7) SMP0000034 map00600
Starch and Sucrose Metabolism (9) SMP0000058 map00500
Steroid Biosynthesis (4) SMP0000023 map00100
Steroidogenesis (3) SMP0000130 map00140
Sulfate/Sulfite Metabolism (2) SMP0000041 map00920
Taurine and Hypotaurine Metabolism (2) SMP0000021 map00430
Thiamine Metabolism (4) SMP0000076 map00730
Threonine and 2-Oxobutanoate Degradation (4) SMP0000452 -
Thyroid Hormone Synthesis (1) SMP0000716 -
Transfer of Acetyl Groups into Mitochondria (9) SMP0000466 -
Trehalose Degradation (3) SMP0000467 -
Tryptophan Metabolism (12) SMP0000063 map00380
Tyrosine Metabolism (11) SMP0000006 map00350
Ubiquinone Biosynthesis (4) SMP0000065 map00130
Urea Cycle (15) SMP0000059 map00330
Valine, Leucine and Isoleucine Degradation (12) SMP0000032 map00280
Vitamin B6 Metabolism (1) SMP0000017 map00750
Vitamin K Metabolism (2) SMP0000464 -
Warburg Effect (22) SMP0000654 -
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