Page last updated: 2024-10-06

Histidine, lysine, phenylalanine, tyrosine, proline and tryptophan catabolism

Proteins (44)

ProteinSynonymsTaxonomy
2-amino-3-carboxymuconate-6-semialdehyde decarboxylaseEC 4.1.1.45; Picolinate carboxylaseHomo sapiens (human)
Glutaryl-CoA dehydrogenase, mitochondrialGCD; EC 1.3.8.6Homo sapiens (human)
Hydroxyproline dehydrogenaseHYPDH; EC 1.5.5.3; Kidney and liver proline oxidase 1; HsPOX1; Probable proline dehydrogenase 2; 1.5.5.2; Probable proline oxidase 2Homo sapiens (human)
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrialP5C dehydrogenase; EC 1.2.1.88; Aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenaseHomo sapiens (human)
Maleylacetoacetate isomeraseMAAI; EC 5.2.1.2; GSTZ1-1; Glutathione S-transferase zeta 1; 2.5.1.18Homo sapiens (human)
Indoleamine 2,3-dioxygenase 1IDO-1; EC 1.13.11.52; Indoleamine-pyrrole 2,3-dioxygenaseHomo sapiens (human)
Indoleamine 2,3-dioxygenase 2IDO-2; EC 1.13.11.-; Indoleamine 2,3-dioxygenase-like protein 1; Indoleamine-pyrrole 2,3-dioxygenase-like protein 1Homo sapiens (human)
Tyrosine aminotransferaseTAT; EC 2.6.1.5; L-tyrosine:2-oxoglutarate aminotransferaseHomo sapiens (human)
Large neutral amino acids transporter small subunit 14F2 light chain; 4F2 LC; 4F2LC; CD98 light chain; Integral membrane protein E16; E16; L-type amino acid transporter 1; hLAT1; Solute carrier family 7 member 5; y+ system cationic amino acid transporterHomo sapiens (human)
KynureninaseEC 3.7.1.3; L-kynurenine hydrolaseHomo sapiens (human)
Kynurenine--oxoglutarate transaminase 3EC 2.6.1.7; Cysteine-S-conjugate beta-lyase 2; 4.4.1.13; Kynurenine aminotransferase 3; Kynurenine aminotransferase III; KATIII; Kynurenine--glyoxylate transaminase; 2.6.1.63; Kynurenine--oxoglutarate transaminase IIIHomo sapiens (human)
Formimidoyltransferase-cyclodeaminaseFormiminotransferase-cyclodeaminase; FTCD; LCHC1Homo sapiens (human)
Histamine N-methyltransferaseHMT; EC 2.1.1.8Homo sapiens (human)
Histidine decarboxylaseHDC; EC 4.1.1.22Homo sapiens (human)
Mitochondrial 2-oxodicarboxylate carrierSolute carrier family 25 member 21Homo sapiens (human)
Tryptophan 2,3-dioxygenaseTDO; EC 1.13.11.11; Tryptamin 2,3-dioxygenase; Tryptophan oxygenase; TO; TRPO; Tryptophan pyrrolase; TryptophanaseHomo sapiens (human)
Kynurenine--oxoglutarate transaminase 1EC 2.6.1.7; Cysteine-S-conjugate beta-lyase; 4.4.1.13; Glutamine transaminase K; GTK; Glutamine--phenylpyruvate transaminase; 2.6.1.64; Kynurenine aminotransferase 1; Kynurenine aminotransferase I; KATI; Kynurenine--oxoglutarate transaminase IHomo sapiens (human)
Kynurenine/alpha-aminoadipate aminotransferase, mitochondrialKAT/AadAT; 2-aminoadipate aminotransferase; 2-aminoadipate transaminase; 2.6.1.39; Alpha-aminoadipate aminotransferase; AadAT; Kynurenine aminotransferase II; Kynurenine--oxoglutarate aminotransferase II; Kynurenine--oxoglutarate transaminase 2; 2.6.1.7; Homo sapiens (human)
Phenylalanine-4-hydroxylasePAH; EC 1.14.16.1; Phe-4-monooxygenaseHomo sapiens (human)
Dihydropteridine reductaseEC 1.5.1.34; HDHPR; Quinoid dihydropteridine reductase; Short chain dehydrogenase/reductase family 33C member 1Homo sapiens (human)
Histidine ammonia-lyaseHistidase; EC 4.3.1.3Homo sapiens (human)
Peroxisomal sarcosine oxidasePSO; EC 1.5.3.1; EC 1.5.3.7; L-pipecolate oxidase; L-pipecolic acid oxidaseHomo sapiens (human)
Kynurenine formamidaseKFA; KFase; EC 3.5.1.9; Arylformamidase; N-formylkynurenine formamidase; FKFHomo sapiens (human)
Carnosine synthase 1EC 6.3.2.11; ATP-grasp domain-containing protein 1Homo sapiens (human)
Kynurenine 3-monooxygenaseEC 1.14.13.9; Kynurenine 3-hydroxylaseHomo sapiens (human)
4-hydroxyphenylpyruvate dioxygenaseEC 1.13.11.27; 4-hydroxyphenylpyruvic acid oxidase; 4HPPD; HPD; HPPDaseHomo sapiens (human)
3-hydroxyanthranilate 3,4-dioxygenaseEC 1.13.11.6; 3-hydroxyanthranilate oxygenase; 3-HAO; h3HAO; 3-hydroxyanthranilic acid dioxygenase; HADHomo sapiens (human)
L-amino-acid oxidaseLAAO; LAO; EC 1.4.3.2; EC 1.4.3.25; Interleukin-4-induced protein 1; IL4-induced protein 1; hIL4I1; Protein Fig-1; hFIG1Homo sapiens (human)
Homogentisate 1,2-dioxygenaseEC 1.13.11.5; Homogentisate oxygenase; Homogentisic acid oxidase; HomogentisicaseHomo sapiens (human)
FumarylacetoacetaseFAA; EC 3.7.1.2; Beta-diketonase; Fumarylacetoacetate hydrolaseHomo sapiens (human)
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialEC 2.3.1.61; 2-oxoglutarate dehydrogenase complex component E2; OGDC-E2; Dihydrolipoamide succinyltransferase component of 2-oxoglutarate dehydrogenase complex; E2KHomo sapiens (human)
Isoaspartyl peptidase/L-asparaginaseEC 3.4.19.5; EC 3.5.1.1; Asparaginase-like protein 1; Beta-aspartyl-peptidase; Isoaspartyl dipeptidase; L-asparagine amidohydrolaseHomo sapiens (human)
Urocanate hydrataseUrocanase; EC 4.2.1.49; Imidazolonepropionate hydrolaseHomo sapiens (human)
Probable imidazolonepropionaseEC 3.5.2.7; Amidohydrolase domain-containing protein 1Homo sapiens (human)
Pterin-4-alpha-carbinolamine dehydratasePHS; EC 4.2.1.96; 4-alpha-hydroxy-tetrahydropterin dehydratase; Dimerization cofactor of hepatocyte nuclear factor 1-alpha; DCoH; Dimerization cofactor of HNF1; Phenylalanine hydroxylase-stimulating protein; Pterin carbinolamine dehydratase; PCDHomo sapiens (human)
Hydroxylysine kinase5-hydroxy-L-lysine kinase; EC 2.7.1.81; Aminoglycoside phosphotransferase domain-containing protein 1Homo sapiens (human)
Proline dehydrogenase 1, mitochondrialEC 1.5.5.2; Proline oxidase; Proline oxidase 2; p53-induced gene 6 proteinHomo sapiens (human)
4F2 cell-surface antigen heavy chain4F2hc; 4F2 heavy chain antigen; Lymphocyte activation antigen 4F2 large subunit; Solute carrier family 3 member 2Homo sapiens (human)
Alpha-aminoadipic semialdehyde dehydrogenaseAlpha-AASA dehydrogenase; EC 1.2.1.31; Aldehyde dehydrogenase family 7 member A1; 1.2.1.3; Antiquitin-1; Betaine aldehyde dehydrogenase; 1.2.1.8; Delta1-piperideine-6-carboxylate dehydrogenase; P6c dehydrogenaseHomo sapiens (human)
Ketimine reductase mu-crystallinEC 1.5.1.25; NADP-regulated thyroid-hormone-binding proteinHomo sapiens (human)
Proton-coupled amino acid transporter 4Proton/amino acid transporter 4; Solute carrier family 36 member 4Homo sapiens (human)
5-phosphohydroxy-L-lysine phospho-lyaseEC 4.2.3.134; Alanine--glyoxylate aminotransferase 2-like 2Homo sapiens (human)
Carnosine N-methyltransferaseEC 2.1.1.22Homo sapiens (human)
Alpha-aminoadipic semialdehyde synthase, mitochondrialLKR/SDHHomo sapiens (human)

Compounds (61)

CompoundDescription
hydronium ion
pyridoxal phosphateThis is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).
pyruvic acidAn intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
Orthophosphate
GlutathioneA tripeptide with many roles in cells. It conjugates to drugs to make them more soluble for excretion, is a cofactor for some enzymes, is involved in protein disulfide bond rearrangement and reduces peroxides.
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
Ammonium
ironA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
NADH
1,5-dihydro-fadchromophore component of E coli DNA photolyase
Aspartate
Adenosine TriphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
fumaric acidsee also record for ferrous fumarate; use FUMARATES for general fumaric acid esters
flavin-adenine dinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
histamineAn amine derived by enzymatic decarboxylation of HISTIDINE. It is a powerful stimulant of gastric secretion, a constrictor of bronchial smooth muscle, a vasodilator, and also a centrally acting neurotransmitter.
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
methanolA colorless, flammable liquid used in the manufacture of FORMALDEHYDE and ACETIC ACID, in chemical synthesis, antifreeze, and as a solvent. Ingestion of methanol is toxic and may cause blindness.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
guanosine diphosphateA guanine nucleotide containing two phosphate groups esterified to the sugar moiety.
guanosine triphosphateGuanosine 5'-(tetrahydrogen triphosphate). A guanine nucleotide containing three phosphate groups esterified to the sugar moiety.
5,6,7,8-tetrahydrofolic acidRN given refers to (DL)-isomer
sapropterinRN given refers to parent cpd; co-factor required for catalytic activity of nitric oxide synthases
4a-hydroxytetrahydrobiopterinstructure given in first source
2-amino-4-(2-aminoethoxy)butanoic acid, (DL)-isomer
3-hydroxykynurenine, (L)-isomer
delta-1-pyrroline-5-carboxylate(L)-isomer is the biologically active form; RN given refers to cpd without isomeric designation; structure
imidazol-4-one-5-propionic acidstructure
saccharopinelysine precursor in the aminoadipic acid-lysine pathway in yeast; minor descriptor (75-85); on-line & Index Medicus search LYSINE/analogs & derivatives (75-85); RN given refers to (L)-isomer
allysinestructure
kynurenineA metabolite of the essential amino acid tryptophan metabolized via the tryptophan-kynurenine pathway.
alpha-Ketoglutarate
glutamic acid gamma-semialdehydestraight-chain form of delta(1)-pyrroline-5-carboxylate; RN given refers to cpd with unspecified isomeric designation
beta-alanineAn amino acid formed in vivo by the degradation of dihydrouracil and carnosine. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, the compound may be a false transmitter replacing GAMMA-AMINOBUTYRIC ACID. A rare genetic disorder, hyper-beta-alaninemia, has been reported.
formic acidRN given refers to parent cpd
glutaryl-coenzyme a
tele-methylhistaminehistamine methyltransferase antagonist
kynurenic acidA broad-spectrum excitatory amino acid antagonist used as a research tool.
pipecolic acidRN given refers to cpd without isomeric designation
3-phenylpyruvate
alpha-ketoglutaric acid
glutamate
crotonyl-coenzyme a
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
acetoacetate
alpha-ketoadipic acid
urocanic acid4-Imidazoleacrylic acid.
indol-3-yl pyruvic acid
3-hydroxyanthranilic acidAn oxidation product of tryptophan metabolism. It may be a free radical scavenger and a carcinogen.
lipoamideRN given refers to cpd without isomeric designation
N'-formylkynureninea photochemical breakdown product of tryptophan in proteins; RN given refers to parent cpd without isomeric designation
anserineA dipeptide containing BETA-ALANINE.
aspartameFlavoring agent sweeter than sugar, metabolized as PHENYLALANINE and ASPARTIC ACID.
s-adenosylhomocysteine5'-S-(3-Amino-3-carboxypropyl)-5'-thioadenosine. Formed from S-adenosylmethionine after transmethylation reactions.
carnosineA naturally occurring dipeptide neuropeptide found in muscles.
hemeThe color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins.
creolinfrom refined coal tar oils
coenzyme a
4-hydroxyphenylpyruvic acidRN given refers to parent cpd