ST001259 Targeted Metabolomic Analysis in Patients with Wilson Disease Reveals Dysregulated Choline, Methionine and Aromatic Amino Acid Metabolism: Implications for Hepatic and Neurological Phenotypes
List of human metabolic pathways and metabolites (REACTOME)

Pathway name (# of metabolites detected in study)Reactome pathway
ABCA transporters in lipid homeostasis (1) REACT_111158
Abnormal metabolism in phenylketonuria (4) REACT_121117
Acetylcholine Neurotransmitter Release Cycle (2) REACT_15309
Acetylcholine regulates insulin secretion (1) REACT_18405
Agmatine biosynthesis (1) REACT_14800
Amino acid synthesis and interconversion (transamination) (3) REACT_238
Amino acid transport across the plasma membrane (11) REACT_13796
Antigen Presentation: Folding, assembly and peptide loading of class I MHC (9) REACT_75795
Antigen processing: Ubiquitination & Proteasome degradation (9) REACT_75842
Aromatic amines can be N-hydroxylated or N-dealkylated by CYP1A2 (1) REACT_13721
Branched-chain amino acid catabolism (2) REACT_197
Carnitine synthesis (1) REACT_2125
Catecholamine biosynthesis (1) REACT_15551
Cell surface interactions at the vascular wall (1) REACT_12051
Cholesterol biosynthesis (1) REACT_9405
Chylomicron-mediated lipid transport (1) REACT_6841
Class A/1 (Rhodopsin-like receptors) (1) REACT_14828
Class C/3 (Metabotropic glutamate/pheromone receptors) (2) REACT_18319
Cleavage of the damaged purine (1) REACT_1729
Cobalamin (Cbl, vitamin B12) transport and metabolism (1) REACT_163862
Conjugation of benzoate with glycine (1) REACT_6933
Creatine metabolism (3) REACT_813
Cross-presentation of soluble exogenous antigens (endosomes) (9) REACT_111056
Cytosolic sulfonation of small molecules (1) REACT_6913
Cytosolic tRNA aminoacylation (9) REACT_15306
Digestion of dietary lipid (2) REACT_9518
Endogenous sterols (2) REACT_13812
Endosomal/Vacuolar pathway (9) REACT_111168
ER-Phagosome pathway (9) REACT_111178
FMO oxidises nucleophiles (1) REACT_13653
G alpha (i) signalling events (3) REACT_19231
G alpha (q) signalling events (2) REACT_18283
G alpha (s) signalling events (1) REACT_19327
Glutathione synthesis and recycling (9) REACT_6960
Glyoxylate metabolism (1) REACT_16925
HDL-mediated lipid transport (1) REACT_13621
Hedgehog ligand biogenesis (1) REACT_228111
Highly calcium permeable nicotinic acetylcholine receptors (1) REACT_22352
Highly calcium permeable postsynaptic nicotinic acetylcholine receptors (1) REACT_22303
Highly sodium permeable acetylcholine nicotinic receptors (1) REACT_22223
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
LDL-mediated lipid transport (1) REACT_6934
Lysosphingolipid and LPA receptors (1) REACT_18365
Metabolism of polyamines (1) REACT_14820
Methionine salvage pathway (1) REACT_75881
Methylation (1) REACT_6946
Miscellaneous substrates (1) REACT_13425
Mitochondrial iron-sulfur cluster biogenesis (1) REACT_150353
Mitochondrial tRNA aminoacylation (9) REACT_15302
Molybdenum cofactor biosynthesis (2) REACT_25073
Muscarinic acetylcholine receptors (1) REACT_16943
Na+/Cl- dependent neurotransmitter transporters (10) REACT_20506
Neurotransmitter Clearance In The Synaptic Cleft (2) REACT_13583
Nicotinamide salvaging (1) REACT_11213
Organic cation transport (3) REACT_22357
Orphan transporters (3) REACT_267716
Passive transport by Aquaporins (1) REACT_23826
Phenylalanine and tyrosine catabolism (3) REACT_1786
Platelet sensitization by LDL (1) REACT_23879
PPARA activates gene expression (1) REACT_116145
Pregnenolone biosynthesis (1) REACT_11038
Proline catabolism (1) REACT_1002
Proton-coupled neutral amino acid transporters (2) REACT_19348
Proton/oligonucleotide cotransporters (9) REACT_19328
Purine catabolism (1) REACT_2086
Purine salvage (1) REACT_1923
Pyrimidine catabolism (1) REACT_1023
Recycling of bile acids and salts (2) REACT_11042
Regulation of cholesterol biosynthesis by SREBP (SREBF) (1) REACT_147797
Retinoid metabolism and transport (1) REACT_24968
Reuptake of GABA (1) REACT_23910
Role of phospholipids in phagocytosis (1) REACT_160158
Scavenging by Class A Receptors (1) 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
Serotonin and melatonin biosynthesis (1) REACT_15439
Sphingolipid de novo biosynthesis (1) REACT_115810
Sulfur amino acid metabolism (2) REACT_115639
Synthesis of bile acids and bile salts (2) REACT_11054
Synthesis of bile acids and bile salts via 24-hydroxycholesterol (1) 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 (3) REACT_11041
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, secretion, and inactivation of Glucagon-like Peptide-1 (GLP-1) (1) REACT_24019
Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation (1) REACT_111176
Thyroxine biosynthesis (2) REACT_15292
Trafficking of dietary sterols (1) REACT_13781
Transport of glucose and other sugars, bile salts and organic acids, metal ions and amine compounds (3) REACT_19305
Transport of nucleosides and free purine and pyrimidine bases across the plasma membrane (1) REACT_1206
Transport of organic anions (1) REACT_23988
Tryptophan catabolism (4) REACT_916
Urea cycle (2) REACT_847
VEGFR2 mediated cell proliferation (1) REACT_228024
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