ST000763 Untargeted metabolomics and lipidomics of scleroderma PAH discovery cohort
List of human metabolic pathways and metabolites (REACTOME)

Pathway name (# of metabolites detected in study)Reactome pathway
5-Phosphoribose 1-diphosphate biosynthesis (1) REACT_850
Abacavir metabolism (1) REACT_121388
ABCA transporters in lipid homeostasis (1) REACT_111158
ABH2 mediated Reversal of Alkylation Damage (1) REACT_526
ABH3 mediated Reversal of Alkylation Damage (1) REACT_1662
Abnormal metabolism in phenylketonuria (4) REACT_121117
Acetylcholine Neurotransmitter Release Cycle (1) REACT_15309
Activation of gene expression by SREBF (SREBP) (4) REACT_147904
Activation of PPARGC1A (PGC-1alpha) by phosphorylation (1) REACT_200686
Activation of the phototransduction cascade (1) REACT_163932
alpha-linolenic acid (ALA) metabolism (2) REACT_121147
Alpha-oxidation of phytanate (2) REACT_17003
Amino acid synthesis and interconversion (transamination) (5) REACT_238
Amino acid transport across the plasma membrane (11) REACT_13796
AMPK inhibits chREBP transcriptional activation activity (1) REACT_1988
Antigen Presentation: Folding, assembly and peptide loading of class I MHC (10) REACT_75795
Antigen processing: Ubiquitination & Proteasome degradation (11) REACT_75842
Arachidonate production from DAG (1) REACT_19308
Arachidonic acid metabolism (1) REACT_147851
Aromatic amines can be N-hydroxylated or N-dealkylated by CYP1A2 (1) REACT_13721
Astrocytic Glutamate-Glutamine Uptake And Metabolism (1) REACT_13639
Biogenic amines are oxidatively deaminated to aldehydes by MAOA and MAOB (1) REACT_416
Biotin transport and metabolism (2) REACT_11153
BMAL1:CLOCK,NPAS2 activates circadian gene expression (4) REACT_111118
Branched-chain amino acid catabolism (2) REACT_197
Ca2+ pathway (1) REACT_172761
Cam-PDE 1 activation (1) REACT_15408
Carnitine synthesis (2) REACT_2125
Catecholamine biosynthesis (2) REACT_15551
Cell surface interactions at the vascular wall (1) REACT_12051
cGMP effects (1) REACT_23767
Cholesterol biosynthesis (3) REACT_9405
Chylomicron-mediated lipid transport (1) REACT_6841
Circadian Clock (4) REACT_24941
Citric acid cycle (TCA cycle) (4) REACT_1785
Class A/1 (Rhodopsin-like receptors) (3) REACT_14828
Class C/3 (Metabotropic glutamate/pheromone receptors) (4) REACT_18319
Class II GLUTs (1) REACT_19281
Cobalamin (Cbl, vitamin B12) transport and metabolism (2) REACT_163862
Coenzyme A biosynthesis (1) REACT_11218
Collagen biosynthesis and modifying enzymes (1) REACT_121139
Condensation of Prophase Chromosomes (2) REACT_172744
Conjugation of benzoate with glycine (3) REACT_6933
Conjugation of phenylacetate with glutamine (2) REACT_6800
Conjugation of salicylate with glycine (3) REACT_6812
COX reactions (1) REACT_1396
Creatine metabolism (2) REACT_813
Cross-presentation of soluble exogenous antigens (endosomes) (10) REACT_111056
CYP2E1 reactions (1) REACT_13797
Cytosolic sulfonation of small molecules (5) REACT_6913
Cytosolic tRNA aminoacylation (11) REACT_15306
DARPP-32 events (1) REACT_15334
Deadenylation of mRNA (1) REACT_20514
degradation of AXIN (1) REACT_200766
Degradation of GABA (1) REACT_23964
Digestion of dietary carbohydrate (1) REACT_9472
Digestion of dietary lipid (4) REACT_9518
DNA methylation (1) REACT_267652
Downregulation of SMAD2/3:SMAD4 transcriptional activity (1) REACT_121111
Endogenous sterols (4) REACT_13812
Endosomal/Vacuolar pathway (10) REACT_111168
eNOS activation (2) REACT_12477
Enzymatic degradation of dopamine by COMT (1) REACT_15548
ER-Phagosome pathway (10) REACT_111178
Ethanol oxidation (1) REACT_34
Factors involved in megakaryocyte development and platelet production (1) REACT_24970
Fatty acids (3) REACT_13814
Fatty Acids bound to GPR40 (FFAR1) regulate insulin secretion (3) REACT_19193
Fatty Acyl-CoA Biosynthesis (4) REACT_1319
FCERI mediated NF-kB activation (1) REACT_163994
formation of the beta-catenin:TCF transactivating complex (1) REACT_200753
Free fatty acid receptors (7) REACT_21268
G alpha (i) signalling events (3) REACT_19231
G alpha (q) signalling events (6) REACT_18283
G alpha (s) signalling events (4) REACT_19327
Glucocorticoid biosynthesis (2) REACT_11036
Gluconeogenesis (4) REACT_1520
Glucuronidation (2) REACT_6784
Glutamate Neurotransmitter Release Cycle (1) REACT_12591
Glutathione synthesis and recycling (11) REACT_6960
Glycogen breakdown (glycogenolysis) (1) REACT_1008
Glycolysis (2) REACT_1383
Glyoxylate metabolism (1) REACT_16925
HDL-mediated lipid transport (1) REACT_13621
HDMs demethylate histones (1) REACT_228178
Hedgehog ligand biogenesis (1) REACT_228111
Heme degradation (1) REACT_22297
Histidine catabolism (1) REACT_1249
Hormone-sensitive lipase (HSL)-mediated triacylglycerol hydrolysis (1) REACT_494
Hydrolysis of LPC (1) REACT_120977
Inactivation, recovery and regulation of the phototransduction cascade (1) REACT_163919
Iron uptake and transport (1) REACT_25060
ISG15 antiviral mechanism (1) REACT_115831
LDL-mediated lipid transport (1) REACT_6934
Ligand-gated ion channel transport (1) REACT_25387
Linoleic acid (LA) metabolism (2) REACT_121100
Lysine catabolism (1) REACT_1298
Meiotic recombination (1) REACT_27271
Metabolism of polyamines (2) REACT_14820
Metabolism of serotonin (1) REACT_15532
Methionine salvage pathway (2) REACT_75881
Methylation (2) REACT_6946
Mineralocorticoid biosynthesis (1) REACT_11047
Miscellaneous substrates (1) REACT_13425
Mismatch repair (MMR) directed by MSH2:MSH3 (MutSbeta) (1) REACT_228019
Mismatch repair (MMR) directed by MSH2:MSH6 (MutSalpha) (1) REACT_228145
Mitochondrial tRNA aminoacylation (11) REACT_15302
Molybdenum cofactor biosynthesis (2) REACT_25073
mRNA Capping (2) REACT_1470
mRNA decay by 3' to 5' exoribonuclease (2) REACT_20619
mRNA decay by 5' to 3' exoribonuclease (2) REACT_20518
Na+/Cl- dependent neurotransmitter transporters (11) REACT_20506
Neurotransmitter Clearance In The Synaptic Cleft (1) REACT_13583
Nicotinamide salvaging (1) REACT_11213
Nicotinate metabolism (2) REACT_11088
Nitric oxide stimulates guanylate cyclase (1) REACT_23862
NoRC negatively regulates rRNA expression (1) REACT_200856
Organic anion transport (1) REACT_22310
Organic anion transporters (1) REACT_19372
Organic cation transport (2) REACT_22357
Orphan transporters (8) REACT_267716
Oxidative Stress Induced Senescence (2) REACT_169436
Oxygen-dependent asparagine hydroxylation of Hypoxia-inducible Factor Alpha (1) REACT_121226
Oxygen-dependent proline hydroxylation of Hypoxia-inducible Factor Alpha (1) REACT_120916
PDE3B signalling (1) REACT_1451
Phagosomal maturation (early endosomal stage) (1) REACT_121256
Phenylalanine and tyrosine catabolism (2) REACT_1786
Phosphate bond hydrolysis by NUDT proteins (1) REACT_150237
phospho-PLA2 pathway (1) REACT_15466
Plasmalogen biosynthesis (1) REACT_1407
Platelet degranulation (1) REACT_318
Platelet sensitization by LDL (1) REACT_23879
PPARA activates gene expression (5) REACT_116145
PRC2 methylates histones and DNA (1) REACT_200808
Pregnenolone biosynthesis (1) REACT_11038
Proline catabolism (1) REACT_1002
Proton-coupled neutral amino acid transporters (1) REACT_19348
Proton/oligonucleotide cotransporters (10) REACT_19328
Purine catabolism (5) REACT_2086
Purine ribonucleoside monophosphate biosynthesis (3) REACT_1776
Purine salvage (3) REACT_1923
Pyrimidine biosynthesis (2) REACT_21376
Pyrimidine catabolism (4) REACT_1023
Pyrimidine salvage reactions (4) REACT_655
Recycling of bile acids and salts (4) REACT_11042
Regulation of AMPK activity via LKB1 (1) REACT_21285
Regulation of cholesterol biosynthesis by SREBP (SREBF) (1) REACT_147797
Regulation of HSF1-mediated heat shock response (1) REACT_200780
Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha) (4) REACT_19241
Regulation of Rheb GTPase activity by AMPK (1) REACT_21393
Regulation of thyroid hormone activity (1) REACT_15297
Removal of the Flap Intermediate (2) REACT_70
Removal of the Flap Intermediate from the C-strand (2) REACT_7999
Respiratory electron transport (1) REACT_22393
Retinoid cycle disease events (1) REACT_160130
Retinoid metabolism and transport (4) REACT_24968
REV-ERBA represses gene expression (4) REACT_118789
RMTs methylate histone arginines (1) REACT_228108
Role of phospholipids in phagocytosis (2) REACT_160158
RORA activates circadian gene expression (4) REACT_118659
Scavenging by Class A Receptors (2) REACT_163699
Scavenging by Class B Receptors (1) REACT_163679
Scavenging by Class F Receptors (1) REACT_163813
Scavenging by Class H Receptors (1) REACT_164002
Scavenging of heme from plasma (1) REACT_160163
Senescence-Associated Secretory Phenotype (SASP) (1) REACT_169168
Serotonin and melatonin biosynthesis (4) REACT_15439
Serotonin clearance from the synaptic cleft (1) REACT_15391
Serotonin Neurotransmitter Release Cycle (1) REACT_15425
Serotonin receptors (1) REACT_17064
SIRT1 negatively regulates rRNA Expression (2) REACT_200827
Sodium-coupled sulphate, di- and tri-carboxylate transporters (2) REACT_20646
Stimuli-sensing channels (2) REACT_160189
Sulfide oxidation to sulfate (1) REACT_116010
Sulfur amino acid metabolism (2) REACT_115639
SUMO is conjugated to E1 (UBA2:SAE1) (1) REACT_163812
Synthesis and interconversion of nucleotide di- and triphosphates (4) REACT_21330
Synthesis of 12-eicosatetraenoic acid derivatives (1) REACT_150201
Synthesis of 15-eicosatetraenoic acid derivatives (1) REACT_150422
Synthesis of (16-20)-hydroxyeicosatetraenoic acids (HETE) (1) REACT_150134
Synthesis of 5-eicosatetraenoic acids (1) REACT_150209
Synthesis of bile acids and bile salts (2) REACT_11054
Synthesis of bile acids and bile salts via 24-hydroxycholesterol (2) REACT_11053
Synthesis of bile acids and bile salts via 27-hydroxycholesterol (2) REACT_11048
Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol (5) REACT_11041
Synthesis of epoxy (EET) and dihydroxyeicosatrienoic acids (DHET) (1) REACT_150417
Synthesis of Hepoxilins (HX) and Trioxilins (TrX) (1) REACT_150292
Synthesis of Leukotrienes (LT) and Eoxins (EX) (1) REACT_150420
Synthesis of PA (1) REACT_120906
Synthesis of PC (2) REACT_121238
Synthesis of PG (1) REACT_121280
Synthesis of Prostaglandins (PG) and Thromboxanes (TX) (1) REACT_150149
Synthesis of PS (1) REACT_120823
Synthesis of UDP-N-acetyl-glucosamine (1) REACT_22394
Synthesis of very long-chain fatty acyl-CoAs (3) REACT_380
Synthesis, secretion, and deacylation of Ghrelin (1) REACT_19189
Synthesis, secretion, and inactivation of Glucagon-like Peptide-1 (GLP-1) (7) REACT_24019
Synthesis, secretion, and inactivation of Glucose-dependent Insulinotropic Polypeptide (GIP) (3) REACT_23824
TET1,2,3 and TDG demethylate DNA (1) REACT_200682
Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation (1) REACT_111176
The canonical retinoid cycle in rods (twilight vision) (3) REACT_160156
The retinoid cycle in cones (daylight vision) (1) REACT_160083
Thyroxine biosynthesis (2) REACT_15292
Trafficking of dietary sterols (1) REACT_13781
Transcriptional activation of mitochondrial biogenesis (4) REACT_200608
Transcriptional regulation of white adipocyte differentiation (4) REACT_27161
Translocation of GLUT4 to the plasma membrane (1) REACT_147867
Transport of fatty acids (8) REACT_23892
Transport of glucose and other sugars, bile salts and organic acids, metal ions and amine compounds (1) REACT_19305
Transport of nucleosides and free purine and pyrimidine bases across the plasma membrane (4) REACT_1206
Transport of nucleotide sugars (1) REACT_22151
Transport of organic anions (3) REACT_23988
Tryptophan catabolism (4) REACT_916
Ubiquinol biosynthesis (1) REACT_160111
Urea cycle (3) REACT_847
Utilization of Ketone Bodies (1) REACT_59
VEGFR2 mediated vascular permeability (1) REACT_228016
Vitamin B1 (thiamin) metabolism (1) REACT_11117
Vitamin B2 (riboflavin) metabolism (1) REACT_11070
Vitamins B6 activation to pyridoxal phosphate (1) REACT_25012
Xenobiotics (1) REACT_13543
YAP1- and WWTR1 (TAZ)-stimulated gene expression (4) REACT_118713
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