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ST002179 Impact of nitisinone on the cerebrospinal fluid metabolome of a murine model of alkaptonuriaList 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 (5)
REACT_121388
ABH2 mediated Reversal of Alkylation Damage (2)
REACT_526
ABH3 mediated Reversal of Alkylation Damage (2)
REACT_1662
Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate (1)
REACT_160183
Abnormal metabolism in phenylketonuria (6)
REACT_121117
Activation of gene expression by SREBF (SREBP) (2)
REACT_147904
Activation of G protein gated Potassium channels (1)
REACT_75831
Activation of PPARGC1A (PGC-1alpha) by phosphorylation (1)
REACT_200686
Adenosine P1 receptors (1)
REACT_18288
Adrenaline,noradrenaline inhibits insulin secretion (1)
REACT_18339
Adrenaline signalling through Alpha-2 adrenergic receptor (1)
REACT_19180
Adrenoceptors (1)
REACT_16927
Agmatine biosynthesis (1)
REACT_14800
alpha-linolenic acid (ALA) metabolism (3)
REACT_121147
Alpha-oxidation of phytanate (4)
REACT_17003
Amino acid synthesis and interconversion (transamination) (10)
REACT_238
Amino acid transport across the plasma membrane (20)
REACT_13796
AMPK inhibits chREBP transcriptional activation activity (1)
REACT_1988
Androgen biosynthesis (1)
REACT_11059
Antigen Presentation: Folding, assembly and peptide loading of class I MHC (17)
REACT_75795
Antigen processing: Ubiquitination & Proteasome degradation (18)
REACT_75842
Aromatic amines can be N-hydroxylated or N-dealkylated by CYP1A2 (1)
REACT_13721
Astrocytic Glutamate-Glutamine Uptake And Metabolism (1)
REACT_13639
Beta oxidation of butanoyl-CoA to acetyl-CoA (1)
REACT_419
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA (1)
REACT_1708
Beta oxidation of hexanoyl-CoA to butanoyl-CoA (1)
REACT_1887
Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA (1)
REACT_735
Beta oxidation of myristoyl-CoA to lauroyl-CoA (1)
REACT_2108
Beta oxidation of octanoyl-CoA to hexanoyl-CoA (1)
REACT_1697
Beta oxidation of palmitoyl-CoA to myristoyl-CoA (1)
REACT_2025
Beta-oxidation of pristanoyl-CoA (1)
REACT_17017
Beta-oxidation of very long chain fatty acids (1)
REACT_17062
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (1)
REACT_22433
Biotin transport and metabolism (2)
REACT_11153
BMAL1:CLOCK,NPAS2 activates circadian gene expression (2)
REACT_111118
Branched-chain amino acid catabolism (5)
REACT_197
Cam-PDE 1 activation (1)
REACT_15408
Carnitine synthesis (4)
REACT_2125
Catecholamine biosynthesis (3)
REACT_15551
Cell redox homeostasis (1)
REACT_121394
Cholesterol biosynthesis (1)
REACT_9405
Circadian Clock (2)
REACT_24941
Citric acid cycle (TCA cycle) (6)
REACT_1785
Class A/1 (Rhodopsin-like receptors) (3)
REACT_14828
Class C/3 (Metabotropic glutamate/pheromone receptors) (6)
REACT_18319
Class II GLUTs (1)
REACT_19281
Cleavage of the damaged purine (2)
REACT_1729
Cleavage of the damaged pyrimidine (2)
REACT_2156
Cobalamin (Cbl, vitamin B12) transport and metabolism (3)
REACT_163862
Coenzyme A biosynthesis (1)
REACT_11218
Collagen biosynthesis and modifying enzymes (2)
REACT_121139
Condensation of Prophase Chromosomes (3)
REACT_172744
Conjugation of benzoate with glycine (2)
REACT_6933
Conjugation of phenylacetate with glutamine (3)
REACT_6800
Conjugation of salicylate with glycine (1)
REACT_6812
Creatine metabolism (6)
REACT_813
Cross-presentation of soluble exogenous antigens (endosomes) (17)
REACT_111056
Cysteine formation from homocysteine (2)
REACT_115589
Cytosolic sulfonation of small molecules (1)
REACT_6913
Cytosolic tRNA aminoacylation (18)
REACT_15306
DARPP-32 events (1)
REACT_15334
Deadenylation of mRNA (1)
REACT_20514
degradation of AXIN (2)
REACT_200766
Degradation of cysteine and homocysteine (4)
REACT_115654
Degradation of GABA (5)
REACT_23964
Digestion of dietary carbohydrate (1)
REACT_9472
Digestion of dietary lipid (2)
REACT_9518
DNA methylation (1)
REACT_267652
Downregulation of SMAD2/3:SMAD4 transcriptional activity (2)
REACT_121111
Endogenous sterols (3)
REACT_13812
Endosomal/Vacuolar pathway (17)
REACT_111168
eNOS activation (3)
REACT_12477
Enzymatic degradation of dopamine by COMT (1)
REACT_15548
ER-Phagosome pathway (17)
REACT_111178
Ethanol oxidation (2)
REACT_34
Factors involved in megakaryocyte development and platelet production (1)
REACT_24970
Fatty acids (2)
REACT_13814
Fatty Acids bound to GPR40 (FFAR1) regulate insulin secretion (3)
REACT_19193
Fatty acid, triacylglycerol, and ketone body metabolism (1)
REACT_22279
Fatty Acyl-CoA Biosynthesis (3)
REACT_1319
FCERI mediated NF-kB activation (1)
REACT_163994
Formation of the active cofactor, UDP-glucuronate (1)
REACT_6737
formation of the beta-catenin:TCF transactivating complex (1)
REACT_200753
Free fatty acid receptors (7)
REACT_21268
Fructose catabolism (1)
REACT_1571
GABA A receptor activation (1)
REACT_24987
GABA A (rho) receptor activation (1)
REACT_25168
GABA B receptor activation (1)
REACT_25031
GABA synthesis (2)
REACT_24020
GABA synthesis, release, reuptake and degradation (1)
REACT_23947
Galactose catabolism (2)
REACT_532
G alpha (12/13) signalling events (1)
REACT_18407
G alpha (i) signalling events (7)
REACT_19231
G alpha (q) signalling events (8)
REACT_18283
G alpha (s) signalling events (5)
REACT_19327
G alpha (z) signalling events (1)
REACT_19333
Glucocorticoid biosynthesis (2)
REACT_11036
Gluconeogenesis (7)
REACT_1520
Glutamate Neurotransmitter Release Cycle (1)
REACT_12591
Glutathione synthesis and recycling (18)
REACT_6960
Glycogen breakdown (glycogenolysis) (1)
REACT_1008
Glycogen synthesis (1)
REACT_169208
Glycolysis (3)
REACT_1383
Glycosphingolipid metabolism (1)
REACT_116105
Glyoxylate metabolism (2)
REACT_16925
HDMs demethylate histones (2)
REACT_228178
Histamine receptors (1)
REACT_16903
Histidine catabolism (5)
REACT_1249
HS-GAG biosynthesis (1)
REACT_121248
HS-GAG degradation (1)
REACT_120752
Hydrolysis of LPC (1)
REACT_120977
Hydrolysis of LPE (1)
REACT_120785
Hypusine synthesis from eIF5A-lysine (2)
REACT_12469
Import of palmitoyl-CoA into the mitochondrial matrix (1)
REACT_11082
Inactivation, recovery and regulation of the phototransduction cascade (1)
REACT_163919
Inhibition of voltage gated Ca2+ channels via Gbeta/gamma subunits (1)
REACT_25004
Inositol transporters (1)
REACT_19188
Interconversion of 2-oxoglutarate and 2-hydroxyglutarate (1)
REACT_25367
Interconversion of polyamines (1)
REACT_14805
ISG15 antiviral mechanism (1)
REACT_115831
Keratan sulfate biosynthesis (1)
REACT_121120
Ligand-gated ion channel transport (1)
REACT_25387
Linoleic acid (LA) metabolism (3)
REACT_121100
Lysine catabolism (3)
REACT_1298
Meiotic recombination (1)
REACT_27271
Metabolism of folate and pterines (1)
REACT_11167
Metabolism of nucleotides (1)
REACT_1698
Metabolism of polyamines (3)
REACT_14820
Metabolism of serotonin (2)
REACT_15532
Methionine salvage pathway (4)
REACT_75881
Methylation (5)
REACT_6946
Mineralocorticoid biosynthesis (2)
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 fatty acid beta-oxidation of unsaturated fatty acids (1)
REACT_160
Mitochondrial iron-sulfur cluster biogenesis (2)
REACT_150353
Mitochondrial tRNA aminoacylation (18)
REACT_15302
Molybdenum cofactor biosynthesis (4)
REACT_25073
mRNA Capping (1)
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 (20)
REACT_20506
NADPH regeneration (1)
REACT_16904
NGF-independant TRKA activation (1)
REACT_11046
Nicotinamide salvaging (1)
REACT_11213
Nicotinate metabolism (3)
REACT_11088
Nitric oxide stimulates guanylate cyclase (2)
REACT_23862
NoRC negatively regulates rRNA expression (1)
REACT_200856
Organic anion transport (2)
REACT_22310
Organic anion transporters (1)
REACT_19372
Organic cation transport (4)
REACT_22357
Orphan transporters (4)
REACT_267716
Oxidative Stress Induced Senescence (3)
REACT_169436
Oxygen-dependent asparagine hydroxylation of Hypoxia-inducible Factor Alpha (2)
REACT_121226
Oxygen-dependent proline hydroxylation of Hypoxia-inducible Factor Alpha (2)
REACT_120916
PAOs oxidise polyamines to amines (1)
REACT_505
PDE3B signalling (1)
REACT_1451
Phagosomal maturation (early endosomal stage) (2)
REACT_121256
Phenylalanine and tyrosine catabolism (6)
REACT_1786
Phosphate bond hydrolysis by NUDT proteins (2)
REACT_150237
Plasmalogen biosynthesis (2)
REACT_1407
PPARA activates gene expression (3)
REACT_116145
PRC2 methylates histones and DNA (1)
REACT_200808
Proline catabolism (2)
REACT_1002
Proton-coupled neutral amino acid transporters (2)
REACT_19348
Proton/oligonucleotide cotransporters (17)
REACT_19328
Purine catabolism (10)
REACT_2086
Purine ribonucleoside monophosphate biosynthesis (5)
REACT_1776
Purine salvage (9)
REACT_1923
Pyrimidine biosynthesis (5)
REACT_21376
Pyrimidine catabolism (10)
REACT_1023
Pyrimidine salvage reactions (7)
REACT_655
Pyruvate metabolism (1)
REACT_2071
Recycling of bile acids and salts (3)
REACT_11042
Regulation of AMPK activity via LKB1 (1)
REACT_21285
Regulation of HSF1-mediated heat shock response (2)
REACT_200780
Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha) (2)
REACT_19241
Regulation of ornithine decarboxylase (ODC) (1)
REACT_13565
Regulation of pyruvate dehydrogenase (PDH) complex (1)
REACT_12528
Regulation of Rheb GTPase activity by AMPK (1)
REACT_21393
Removal of the Flap Intermediate (2)
REACT_70
Removal of the Flap Intermediate from the C-strand (2)
REACT_7999
Respiratory electron transport (2)
REACT_22393
Retinoid cycle disease events (2)
REACT_160130
Retinoid metabolism and transport (3)
REACT_24968
Reuptake of GABA (1)
REACT_23910
REV-ERBA represses gene expression (2)
REACT_118789
RMTs methylate histone arginines (1)
REACT_228108
RORA activates circadian gene expression (2)
REACT_118659
Scavenging by Class A Receptors (1)
REACT_163699
Senescence-Associated Secretory Phenotype (SASP) (1)
REACT_169168
Serine biosynthesis (4)
REACT_115789
Serotonin and melatonin biosynthesis (2)
REACT_15439
SIRT1 negatively regulates rRNA Expression (3)
REACT_200827
Sodium-coupled sulphate, di- and tri-carboxylate transporters (2)
REACT_20646
Sphingolipid de novo biosynthesis (3)
REACT_115810
Stimuli-sensing channels (2)
REACT_160189
Sulfide oxidation to sulfate (1)
REACT_116010
Sulfur amino acid metabolism (4)
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 15-eicosatetraenoic acid derivatives (1)
REACT_150422
Synthesis of bile acids and bile salts (1)
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 (4)
REACT_11041
Synthesis of IP2, IP, and Ins in the cytosol (3)
REACT_150352
Synthesis of Ketone Bodies (1)
REACT_1464
Synthesis of Lipoxins (LX) (1)
REACT_150320
Synthesis of PA (3)
REACT_120906
Synthesis of PC (1)
REACT_121238
Synthesis of PE (1)
REACT_120919
Synthesis of PG (1)
REACT_121280
Synthesis of PI (1)
REACT_121000
Synthesis of Prostaglandins (PG) and Thromboxanes (TX) (1)
REACT_150149
Synthesis of PS (1)
REACT_120823
Synthesis of UDP-N-acetyl-glucosamine (2)
REACT_22394
Synthesis of very long-chain fatty acyl-CoAs (2)
REACT_380
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 (2)
REACT_200682
Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation (1)
REACT_111176
The activation of arylsulfatases (1)
REACT_121036
The canonical retinoid cycle in rods (twilight vision) (4)
REACT_160156
The retinoid cycle in cones (daylight vision) (1)
REACT_160083
Thyroxine biosynthesis (2)
REACT_15292
Tolerance by Mtb to nitric oxide produced by macrophages (1)
REACT_120983
Transcriptional activation of mitochondrial biogenesis (2)
REACT_200608
Transcriptional regulation of white adipocyte differentiation (2)
REACT_27161
Translocation of GLUT4 to the plasma membrane (1)
REACT_147867
Transport of fatty acids (5)
REACT_23892
Transport of glucose and other sugars, bile salts and organic acids, metal ions and amine compounds (1)
REACT_19305
Transport of inorganic cations/anions and amino acids/oligopeptides (3)
REACT_19397
Transport of nucleosides and free purine and pyrimidine bases across the plasma membrane (12)
REACT_1206
Transport of nucleotide sugars (1)
REACT_22151
Transport of organic anions (1)
REACT_23988
Triglyceride Biosynthesis (2)
REACT_1190
Tryptophan catabolism (6)
REACT_916
Ubiquinol biosynthesis (1)
REACT_160111
Urea cycle (5)
REACT_847
Utilization of Ketone Bodies (2)
REACT_59
VEGFR2 mediated vascular permeability (2)
REACT_228016
Vitamin B1 (thiamin) metabolism (1)
REACT_11117
Vitamin B2 (riboflavin) metabolism (2)
REACT_11070
Vitamin C (ascorbate) metabolism (1)
REACT_11202
Vitamins (1)
REACT_13450
Vitamins B6 activation to pyridoxal phosphate (4)
REACT_25012
YAP1- and WWTR1 (TAZ)-stimulated gene expression (2)
REACT_118713
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