#METABOLOMICS WORKBENCH juliehaines_20250310_124425 DATATRACK_ID:5714 STUDY_ID:ST003794 ANALYSIS_ID:AN006236 PROJECT_ID:PR002369
VERSION             	1
CREATED_ON             	March 13, 2025, 7:40 pm
#PROJECT
PR:PROJECT_TITLE                 	Advancing the Biochemical Understanding of Maple Syrup Urine Disease and the
PR:PROJECT_TITLE                 	impact of liver transplantation: A Pilot Study
PR:PROJECT_SUMMARY               	Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic
PR:PROJECT_SUMMARY               	disorder caused by impaired catabolism of branched-chain amino acids (BCAAs),
PR:PROJECT_SUMMARY               	leading to severe systemic dysregulation. The disease has an incidence of
PR:PROJECT_SUMMARY               	approximately 1 in 185,000 live births in the general U.S. population, with much
PR:PROJECT_SUMMARY               	higher prevalence in the Mennonite communities (up to 1 in 140 live births in
PR:PROJECT_SUMMARY               	the latter due to the c.1312T>A p.Tyr438Asn BCKDHA founder mutation). Using a
PR:PROJECT_SUMMARY               	multi-omics approach integrating metabolomics, lipidomics, and proteomics, we
PR:PROJECT_SUMMARY               	analyzed blood samples from three MSUD patients on a BCAA-restricted diet, two
PR:PROJECT_SUMMARY               	post-liver transplantation patients, and six healthy controls. Gene ontology
PR:PROJECT_SUMMARY               	analysis highlighted enriched pathways in MSUD, including glycolysis, oxidative
PR:PROJECT_SUMMARY               	phosphorylation, and purine metabolism, revealing systemic metabolic imbalances.
PR:PROJECT_SUMMARY               	Lipidomics indicated disruptions in sphingolipids and lysophosphatidylcholines,
PR:PROJECT_SUMMARY               	which impact cellular signaling and membrane integrity. Liver transplantation
PR:PROJECT_SUMMARY               	corrected some abnormalities, but several metabolites and proteins remained
PR:PROJECT_SUMMARY               	dysregulated. Proteomic analysis revealed significant alterations in redox
PR:PROJECT_SUMMARY               	homeostasis, energy metabolism, and cytoskeletal organization, with only partial
PR:PROJECT_SUMMARY               	recovery post-transplantation. Post-translational modifications, such as
PR:PROJECT_SUMMARY               	methylation and cysteine oxidation, suggested ongoing oxidative stress and
PR:PROJECT_SUMMARY               	immune activation in the LT group. Elevated levels of L-isoleucine, L-valine,
PR:PROJECT_SUMMARY               	and their ketoacids persisted post-transplant, correlating with impaired amino
PR:PROJECT_SUMMARY               	acid metabolism, lipid remodeling, and protein folding. These findings provide
PR:PROJECT_SUMMARY               	comprehensive insight into MSUD-associated metabolic dysfunctions and highlight
PR:PROJECT_SUMMARY               	potential therapeutic targets to improve patient outcomes.
PR:INSTITUTE                     	University of Colorado Denver
PR:LABORATORY                    	Angelo D'Alessandro in collaboration with D. Holmes Morton
PR:LAST_NAME                     	Haines
PR:FIRST_NAME                    	Julie
PR:ADDRESS                       	12801 E 17th Ave, Room 1303, Aurora, Colorado, 80045, USA
PR:EMAIL                         	julie.haines@cuanschutz.edu
PR:PHONE                         	3037243339
#STUDY
ST:STUDY_TITLE                   	Metabolomics characterization of blood samples from patients with maple syrup
ST:STUDY_TITLE                   	urine disease (MSUD) in comparison to healthy controls
ST:STUDY_SUMMARY                 	Metabolomics analysis was performed on blood samples from MSUD patients and MSUD
ST:STUDY_SUMMARY                 	patients post-liver transplantation in comparison to healthy controls.
ST:INSTITUTE                     	University of Colorado Denver
ST:LAST_NAME                     	Haines
ST:FIRST_NAME                    	Julie
ST:ADDRESS                       	12801 E 17th Ave, Room 1303, Aurora, Colorado, 80045, USA
ST:EMAIL                         	julie.haines@cuanschutz.edu
ST:PHONE                         	3037243339
#SUBJECT
SU:SUBJECT_TYPE                  	Human
SU:SUBJECT_SPECIES               	Homo sapiens
SU:TAXONOMY_ID                   	9606
SU:GENDER                        	Male and female
#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           	-	AG-88-1	Disease state:Healthy control | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-1
SUBJECT_SAMPLE_FACTORS           	-	AG-88-2	Disease state:Healthy control | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-2
SUBJECT_SAMPLE_FACTORS           	-	AG-88-3	Disease state:Healthy control | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-3
SUBJECT_SAMPLE_FACTORS           	-	AG-88-4	Disease state:Healthy control | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-4
SUBJECT_SAMPLE_FACTORS           	-	AG-88-5	Disease state:Healthy control | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-5
SUBJECT_SAMPLE_FACTORS           	-	AG-88-6	Disease state:Healthy control | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-6
SUBJECT_SAMPLE_FACTORS           	-	AG-88-7	Disease state:MSUD | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-7
SUBJECT_SAMPLE_FACTORS           	-	AG-88-8	Disease state:MSUD | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-8
SUBJECT_SAMPLE_FACTORS           	-	AG-88-9	Disease state:MSUD | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-9
SUBJECT_SAMPLE_FACTORS           	-	AG-88-10	Disease state:MSUD with transplant | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-10
SUBJECT_SAMPLE_FACTORS           	-	AG-88-11	Disease state:MSUD with transplant | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-11
SUBJECT_SAMPLE_FACTORS           	-	AG-88-12	Disease state:Pool of MSUD with transplant | Sample source:Whole blood	RAW_FILE_NAME(raw file name)=AG-88-12
#COLLECTION
CO:COLLECTION_SUMMARY            	The study was conducted in accordance with the Declaration of Helsinki;
CO:COLLECTION_SUMMARY            	specimens were collected at the Central Pennsylvania Clinic, Boston Children’s
CO:COLLECTION_SUMMARY            	Hospital, and Lancaster General Hospitals under institutionally reviewed
CO:COLLECTION_SUMMARY            	Protocol No. 2014-12 and upon signing of informed consent. Blood collections
CO:COLLECTION_SUMMARY            	were carried out using standard equipment and clinical procedures. To study the
CO:COLLECTION_SUMMARY            	metabolic changes occurring in patients with maple syrup urine disease, whole
CO:COLLECTION_SUMMARY            	blood was collected from 3 patients having diagnosis of MSUD with a biallelic
CO:COLLECTION_SUMMARY            	inactivating mutation of BCKDHA (c.1312T>A p.Tyr438Asn). Additional samples were
CO:COLLECTION_SUMMARY            	collected from 6 healthy controls. Finally, samples were collected from two
CO:COLLECTION_SUMMARY            	patients having a previous diagnosis of MSUD, but who had received liver
CO:COLLECTION_SUMMARY            	transplantations to cure their condition. These two samples were combined 1:1,
CO:COLLECTION_SUMMARY            	v:v, to generate a third replicate for statistical analysis.
CO:SAMPLE_TYPE                   	Blood (whole)
#TREATMENT
TR:TREATMENT_SUMMARY             	No treatment was performed
#SAMPLEPREP
SP:SAMPLEPREP_SUMMARY            	Blood aliquots were thawed on ice, then 10 uL of whole blood was treated with
SP:SAMPLEPREP_SUMMARY            	with 90 uL of cold 5:3:2 methanol:acetonitrile:water. Samples were vortexed 30
SP:SAMPLEPREP_SUMMARY            	min at 4 degrees C then supernatants clarified by centrifugation (10 min, 10,000
SP:SAMPLEPREP_SUMMARY            	g, 4 degrees C). For negative ion mode runs, supernatants were transferred to
SP:SAMPLEPREP_SUMMARY            	autosampler vials. For positive ion mode runs, 35 uL of extract was dried on a
SP:SAMPLEPREP_SUMMARY            	speedvac then resuspended in 35 uL of 0.1% formic acid in water. FA solutions
SP:SAMPLEPREP_SUMMARY            	were then transferred to autosampler vials for analysis.
SP:PROCESSING_STORAGE_CONDITIONS 	4℃
SP:EXTRACT_STORAGE               	-80℃
#CHROMATOGRAPHY
CH:CHROMATOGRAPHY_SUMMARY        	Negative C18
CH:CHROMATOGRAPHY_TYPE           	Reversed phase
CH:INSTRUMENT_NAME               	Thermo Vanquish
CH:COLUMN_NAME                   	Waters ACQUITY UPLC BEH C18 (100 x 2.1mm,1.7um)
CH:SOLVENT_A                     	5% acetonitrile/95% water; 5 mM ammonium acetate
CH:SOLVENT_B                     	95% acetonitrile/5% water; 5 mM ammonium acetate
CH:FLOW_GRADIENT                 	0-0.5 min hold at 0% B, 0.5-1.1 min increase to 100% B, 1.1-2.75 min hold at
CH:FLOW_GRADIENT                 	100% B, 2.75-3 min decrease to 0% B, 3-5 min hold at 0% B
CH:FLOW_RATE                     	0.45 mL/min
CH:COLUMN_TEMPERATURE            	45
CH:SAMPLE_INJECTION              	10 uL
#ANALYSIS
AN:ANALYSIS_TYPE                 	MS
#MS
MS:INSTRUMENT_NAME               	Thermo Orbitrap Exploris 120
MS:INSTRUMENT_TYPE               	Orbitrap
MS:MS_TYPE                       	ESI
MS:ION_MODE                      	NEGATIVE
MS:MS_COMMENTS                   	The Q Exactive was run in negative ion mode, scanning using full MS from 65-975
MS:MS_COMMENTS                   	m/z at 120,000 resolution. Electrospray ionization was achieved with 50 Arb
MS:MS_COMMENTS                   	sheath gas, 10 Arb auxiliary gas, and 3 kV spray voltage. Calibration was
MS:MS_COMMENTS                   	performed prior to the run using the PierceTM Positive and Negative Ion
MS:MS_COMMENTS                   	Calibration Solutions (Thermo Fisher Scientific). Run order of samples was
MS:MS_COMMENTS                   	randomized and technical replicates were injected after every 4 samples to
MS:MS_COMMENTS                   	assess quality control.
#MS_METABOLITE_DATA
MS_METABOLITE_DATA:UNITS	peak area
MS_METABOLITE_DATA_START
Samples	AG-88-1	AG-88-2	AG-88-3	AG-88-4	AG-88-5	AG-88-6	AG-88-7	AG-88-8	AG-88-9	AG-88-10	AG-88-11	AG-88-12
Factors	Disease state:Healthy control | Sample source:Whole blood	Disease state:Healthy control | Sample source:Whole blood	Disease state:Healthy control | Sample source:Whole blood	Disease state:Healthy control | Sample source:Whole blood	Disease state:Healthy control | Sample source:Whole blood	Disease state:Healthy control | Sample source:Whole blood	Disease state:MSUD | Sample source:Whole blood	Disease state:MSUD | Sample source:Whole blood	Disease state:MSUD | Sample source:Whole blood	Disease state:MSUD with transplant | Sample source:Whole blood	Disease state:MSUD with transplant | Sample source:Whole blood	Disease state:Pool of MSUD with transplant | Sample source:Whole blood
glycine	466614	546020	411829	430852	434297	499400	799135	525566	717210	578105	599232	704481
4-Methyl-2-Oxpentanoate/(S)-3-Methyl-2-Oxopentanoic Acid (ketoacids of Ile and Leu)	3979470	2465150	3542359	2342348	4903095	4710568	8724376	44493708	16774944	12342003	34239404	22105800
3-Methyl-2-Oxobutanoate (ketoacid of Val)	285402	186107	223336	186783	310081	244396	526609	1117966	1181681	599505	1073736	760161
L-cystine	113895	140508	261614	235200	363109	103855	112324	126120	40553	181699	86103	135267
ATP	3532202	2703264	3291713	2164781	2526715	1453303	69333	2452231	1756146	1151121	1826653	1504946
ADP	7140995	5302198	5668330	7519953	7188327	6906394	2472224	8414191	5327123	6609845	7824236	7579892
dAMP	47258	224128	35183	84926	241167	249138	108339	93782	178704	72793	121683	60481
GTP	21083	19393	65880	9554	101803	8519	0	7127	13753	7337	11883	6075
GDP	152677	348712	101474	92099	158576	308603	229663	192730	683230	98631	236332	183631
CDP	14309	35787	25913	25895	61996	11857	14746	9049	8052	21540	5946	23479
Cytidine	136792	67906	138710	126655	77637	89640	15966	41071	12036	41302	13386	23974
UTP	23688	138917	292157	164959	196472	51341	0	142805	131262	89660	94198	92525
UDP	2795835	2213462	3046668	2806685	5664696	4125783	2170798	2359892	974574	4368987	2142563	3197919
IDP	150941	204303	197308	236159	218913	281795	210996	200872	168508	156208	174115	217687
IMP	26398032	44412288	27989568	37411340	19303130	28428614	46200252	41046508	44048892	41148404	47130848	41653520
Urate	16737375	15935768	24884840	18073896	29559488	20771928	29040586	23763530	31844390	27577718	20629360	24371250
3',5'-Cyclic IMP	4876240	4457984	6575490	6403547	7966091	5505051	4798955	5397751	2524015	5991496	4755628	5712591
5'-Phosphoribosyl-N-formylglycinamide	55275	107715	215893	228179	206185	111973	0	96285	50612	215181	173663	188338
Pyridoxal	9297	10129	10933	11225	11169	12588	11925	35108	20447	9479	381934	219535
4-Pyridoxate	254312	91157	84035	100045	112906	135272	87999	756910	59383	71342	7594639	3700703
Pyridoxamine 5'-phosphate	22245	196320	31523	137021	119528	10065	80671	104470	180077	54766	32157	55885
Nicotinate ribonucleotide	452163	235717	720470	543039	1137387	397373	328635	257204	39062	395286	141160	275032
UDP-glucose	152928	137662	87112	102661	65029	116330	102302	177471	199330	97787	157730	117753
NADP+	456869	217356	326462	336599	273284	291170	328657	409071	404503	348965	365780	322684
Phosphate	21757170	28021094	17743658	24721688	24130946	30267298	35360272	27880870	29992534	27374106	34025436	28652206
Diphosphate	677858	1080047	955602	1324391	1109779	2000109	2972236	1039514	1553971	964610	1360301	1072717
D-Glucose	20791242	13201824	16033291	10994628	20579578	21409488	19039244	14360365	13817011	22822170	14526976	17709482
D-Glucose 6-phosphate	2662528	3131834	2516401	2123761	1388508	1870145	4985709	5049560	3618644	3383498	3128733	3482714
D-Fructose 1-6-bisphosphate	16327531	37603868	12461575	26016416	17195844	14936541	35761136	28379200	34530056	36809204	22730672	28418752
D-Glyceraldehyde 3-phosphate/Glycerone phosphate	4566206	6932574	3211095	4913172	3949267	4163356	9098933	6976719	9022806	8257012	10157501	9678907
1-3-Bisphosphoglycerate	84719936	83098536	80710696	80374248	103715096	55919824	51801644	76862544	53466876	92354752	50799616	69033744
2/3-Phospho-D-glycerate	4735806	2303639	3905103	3640334	4497438	2927349	1793503	1835837	895634	2288960	1050425	1511168
Phosphoenolpyruvate	6591192	6119639	5791106	6104623	7485054	4270332	3777309	5685996	3760162	6547730	3490534	4889670
Pyruvate	2947825	9407305	3542799	6604818	3764191	6393598	15770455	9597808	13189813	5425212	11690163	8273352
Lactate	31054358	39022084	66869780	60138628	48247940	26240326	36559136	44078604	57426740	40377840	46689900	41047692
Maltose	103994	619697	811351	811607	639606	771762	957621	572229	380212	832936	820127	877102
Maltotriose	41941	370167	627288	339596	404732	205659	139351	497708	183894	474810	294025	411998
Mannitol	1204598	2343608	1770368	1203524	1020741	2176087	3256531	2260296	2880361	2176138	1610624	1909841
D-Ribose	890887	586781	652741	436358	846484	1028459	635538	670000	577134	915916	434864	749256
D-Rhamnose	473394	360405	715710	434689	565704	511013	328118	566879	397009	223541	426554	307299
D-Arabitol	213434	481569	178057	348385	313857	383750	365560	201341	205249	202371	244462	228999
Citrate	14058904	6198444	61422996	37199788	89383912	20024466	9437557	12915979	6665404	18337520	7974246	12899320
2-Oxoglutarate	2235869	603387	2334044	3882578	613324	1168627	1001824	4380410	143580	2585040	4572503	3116727
Succinate	592007	789093	779651	1246010	973890	875327	814228	715108	672211	486494	1040462	655436
Fumarate	986434	1605354	1322232	1294586	1191729	967550	1269696	995588	1521018	1229974	1585840	1216496
Malate	16864386	26071638	21626210	22140018	20919762	15170181	17733916	18945470	23815530	20091490	25338574	19884806
2-Hydroxyglutarate/Citramalate	2740842	4097557	3053519	4008814	3612112	2841457	3095371	3797785	3899338	2502681	3476848	2409449
6-Phospho-D-gluconate	656707	956842	762575	618375	512679	705721	594830	1016489	1393662	692162	791579	714505
D-Erythrose 4-phosphate	82467	59657	43877	10690	34349	40457	44474	80452	67177	21458	42806	38554
Sedoheptulose 1-phosphate	83878	181003	40716	88608	59964	125847	136750	139632	298816	134909	126076	114412
alpha-D-Ribose 1-phosphate	1090719	5594320	622395	1028958	1463650	1227393	2516848	3239717	7278613	3590260	3051519	2954641
5-Oxoproline	2709873	4509018	4156629	5147067	4403662	3175966	5224536	4087878	7590486	5458920	4864611	5494521
N-Acetylneuraminate	1002946	1622657	1332872	1568537	1324178	1397641	1577492	2019379	2140271	1550579	2981740	1996734
1-4-beta-D-Xylan	690463	423927	617573	432747	793564	719254	744973	611749	488757	958686	597068	690993
UDP-N-acetyl-D-glucosamine	202802	68190	150770	93123	131487	64482	124524	212095	441080	61848	126061	151123
N-Acetylornithine	115120	254359	220729	125975	140475	276183	128107	96409	300196	140681	162345	133282
trans-4-Hydroxy-L-proline	295819	483084	575963	487024	586577	456049	614089	337562	636425	457268	518104	540941
N-Succinyl-L-glutamate 5-semialdehyde	255743	554303	576152	521979	644375	238863	584022	424994	351083	260408	624500	415849
3-Sulfocatechol	3453954	3448242	1381209	5586090	4501252	5990082	538238	2245730	3676940	977140	2171515	1400257
5-Hydroxyindoleacetate	121859	415905	342847	449028	197878	190075	928811	559394	1070462	331146	655068	480114
3-Methyleneoxindole	2177520	1130355	1601923	1474540	439546	773324	978204	1035037	762949	1657221	1012539	1178015
Indole-3-acetate	56384	44828	40490	25415	39765	55341	43136	49581	18622	31350	40650	36069
2-Aminomuconate	400560	427269	393353	519609	665750	475419	688916	432917	469572	704680	468526	527652
Quinolinic Acid	34778	120505	120609	71559	73075	62492	67618	83655	165186	79476	77171	45416
Glycerol 3-phosphate	865573	710630	180999	171893	633733	748335	241863	688478	922657	579955	436022	536885
Ethanolamine phosphate	24281	245420	493575	473213	275414	142727	327700	333232	336310	294669	439446	352886
Sphingosine 1-phosphate	289890	419823	404328	478711	317161	468673	482796	665989	575383	755048	1064698	779048
Heptanoic acid	62645	35273	67513	50876	36195	35587	40451	38830	36180	58116	24462	29081
Octanoic acid (caprylate)	111904	92004	157917	126293	147844	99764	85977	85623	80591	119668	125709	117751
Nonanoic acid (pelargonate)	501795	488052	708751	664966	585522	688759	513909	574658	506683	671714	645473	571572
Hexadecanoic acid (palmitic acid)	252858096	290259712	425453536	323784704	377684000	383187232	262274960	402803232	207397712	282437888	242928912	251681856
Tetradecenoic acid (myristoleic acid)	376880	726349	1385447	319702	5784196	1267835	751150	845547	309990	437125	276357	318802
Octadecenoic acid (Oleic acid)	107957080	159055088	190661520	78609608	481994784	245082512	229081904	169607184	96811560	81528544	87401336	84808888
Linoleic acid ((9Z,12Z)-Octadecadienoic acid)	54452304	113862144	84841000	42562884	312265184	247701824	154881968	155192576	71359512	41396048	85657080	59915888
a-Linolenic acid (Octadecatrienoic acid)	2490233	6403652	5026770	4109901	22903792	23867078	6600468	14548349	5155651	2730231	6677708	4535530
(8Z-11Z-14Z)-Icosatrienoic acid	1932173	5485629	4590310	3183671	8088771	5132096	6417316	6055853	4139024	3112656	3373178	3012484
Hemine	95130	99083	97725	83854	89697	92259	77850	92378	62874	103446	83179	73685
MS_METABOLITE_DATA_END
#METABOLITES
METABOLITES_START
metabolite_name	KEGG ID	Pathway	m/z	r.t.
glycine	C00037	Amino acids	74.0248	0.4910
4-Methyl-2-Oxpentanoate/(S)-3-Methyl-2-Oxopentanoic Acid (ketoacids of Ile and Leu)	C00233/C00671	Amino acids	129.0557	1.6460
3-Methyl-2-Oxobutanoate (ketoacid of Val)	C00141	Amino acids	115.0401	0.6370
L-cystine	C00491	Amino acids	239.0149	0.4930
ATP	C00002	Nucleotides	505.988525	0.529
ADP	C00008	Nucleotides	426.022308	0.537
dAMP	C00360	Nucleotides	330.055817	0.533
GTP	C00044	Nucleotides	521.984436	0.456
GDP	C00035	Nucleotides	442.010223	0.529
CDP	C00112	Nucleotides	402.00766	1.698
Cytidine	C00475	Nucleotides	242.079117	0.496
UTP	C00075	Nucleotides	482.961243	0.447
UDP	C00015	Nucleotides	402.999023	0.481
IDP	C00104	Nucleotides	427.005798	0.46
IMP	C00130	Nucleotides	347.039734	0.531
Urate	C00366	Nucleotides	167.021042	0.532
3',5'-Cyclic IMP	C00943	Nucleotides	329.030457	0.481
5'-Phosphoribosyl-N-formylglycinamide	C04376	Nucleotides	313.040283	0.494
Pyridoxal	C00250	Nucleotides	166.050995	0.617
4-Pyridoxate	C00847	Nucleotides	182.045898	1.621
Pyridoxamine 5'-phosphate	C00647	Nucleotides	247.044754	0.558
Nicotinate ribonucleotide	C01185	Nucleotides	335.034821	0.56
UDP-glucose	C00029	Nucleotides	565.048218	0.486
NADP+	C00006	Nucleotides	742.068787	0.526
Phosphate	C00009	Phosphates	96.96962	0.465
Diphosphate	C00013	Phosphates	176.935654	0.459
D-Glucose	C00031	Glycolysis	179.056137	0.503
D-Glucose 6-phosphate	C02965	Glycolysis	259.022552	0.458
D-Fructose 1-6-bisphosphate	C00354	Glycolysis	338.988708	0.43
D-Glyceraldehyde 3-phosphate/Glycerone phosphate	C00118	Glycolysis	168.990829	0.46
1-3-Bisphosphoglycerate	C00236	Glycolysis	264.951965	0.427
2/3-Phospho-D-glycerate	C00631	Glycolysis	184.985306	0.443
Phosphoenolpyruvate	C00074	Glycolysis	166.975098	0.427
Pyruvate	C00022	Glycolysis	87.008766	0.522
Lactate	C01432	Glycolysis	89.024406	0.497
Maltose	C00208	Other sugars	341.108917	0.539
Maltotriose	C01835	Other sugars	503.161957	0.536
Mannitol	C00392	Other sugars	181.071182	0.513
D-Ribose	C00121	Other sugars	149.045639	0.505
D-Rhamnose	C01684	Other sugars	163.061234	0.545
D-Arabitol	C01904	Other sugars	151.061234	0.549
Citrate	C00158	TCA cycle	191.01976	0.43
2-Oxoglutarate	C00026	TCA cycle	145.014618	0.459
Succinate	C00042	TCA cycle	117.019402	0.46
Fumarate	C00122	TCA cycle	115.00367	0.474
Malate	C00149	TCA cycle	133.014236	0.461
2-Hydroxyglutarate/Citramalate	C02630	Alternative Carboxylic acids	147.030106	0.462
6-Phospho-D-gluconate	C00345	Pentose Phosphate Pathway	275.017395	0.436
D-Erythrose 4-phosphate	C00279	Pentose Phosphate Pathway	199.001312	0.51
Sedoheptulose 1-phosphate	C06222	Pentose Phosphate Pathway	289.033081	0.46
alpha-D-Ribose 1-phosphate	C00620	Pentose Phosphate Pathway	229.011871	0.465
5-Oxoproline	C01879	GSH homeostasis	128.035324	0.527
N-Acetylneuraminate	C00270	Aminosugars	308.098785	0.515
1-4-beta-D-Xylan	C02352	Aminosugars	131.034973	0.49
UDP-N-acetyl-D-glucosamine	C00043	Aminosugars	606.074463	0.503
N-Acetylornithine	C00437	Arginine and proline metabolism	173.093185	0.543
trans-4-Hydroxy-L-proline	C01157	Arginine and proline metabolism	130.050995	0.535
N-Succinyl-L-glutamate 5-semialdehyde	C05932	Arginine and proline metabolism	230.063644	0.498
3-Sulfocatechol	C06336	Sulfur metabolism	188.986221	1.643
5-Hydroxyindoleacetate	C05635	Indole and Tryptophan	190.054001	0.682
3-Methyleneoxindole	C02796	Indole and Tryptophan	144.045471	1.805
Indole-3-acetate	C00954	Indole and Tryptophan	174.055466	1.669
2-Aminomuconate	C02220	Indole and Tryptophan	156.028427	0.529
Quinolinic Acid	C03722	Indole and Tryptophan	166.014526	0.537
Glycerol 3-phosphate	C00093	Glycerophospholipid biosynthesis	171.007324	0.46
Ethanolamine phosphate	C00346	Glycerophospholipid biosynthesis	140.010803	0.493
Sphingosine 1-phosphate	C06124	Glycerophospholipid biosynthesis	378.241577	2.292
Heptanoic acid	C17714	Saturated Fatty acids	129.092133	1.828
Octanoic acid (caprylate)	C06423	Saturated Fatty acids	143.107758	1.892
Nonanoic acid (pelargonate)	C01601	Saturated Fatty acids	157.123474	1.966
Hexadecanoic acid (palmitic acid)	C00249	Saturated Fatty acids	255.232864	2.553
Tetradecenoic acid (myristoleic acid)	C08322	Monounsaturated Fatty Acids	225.18602	2.221
Octadecenoic acid (Oleic acid)	C00712	Monounsaturated Fatty Acids	281.248535	2.563
Linoleic acid ((9Z,12Z)-Octadecadienoic acid)	C01595	Poly-unsaturated Fatty Acids	279.23291	2.398
a-Linolenic acid (Octadecatrienoic acid)	C06427	Poly-unsaturated Fatty Acids	277.217316	2.292
(8Z-11Z-14Z)-Icosatrienoic acid	C03242	Essential fatty acids	305.248657	2.449
Hemine	C06767	Hemolysis markers	650.139099	2.233
METABOLITES_END
#END