Page last updated: 2024-10-06

Hawkinsinuria

Proteins (16)

ProteinSynonymsTaxonomy
Amine oxidase [flavin-containing] AEC 1.4.3.4; Monoamine oxidase type A; MAO-AHomo sapiens (human)
Maleylacetoacetate isomeraseMAAI; EC 5.2.1.2; GSTZ1-1; Glutathione S-transferase zeta 1; 2.5.1.18Homo sapiens (human)
Macrophage migration inhibitory factorMIF; EC 5.3.2.1; Glycosylation-inhibiting factor; GIF; L-dopachrome isomerase; L-dopachrome tautomerase; 5.3.3.12; Phenylpyruvate tautomeraseHomo sapiens (human)
TyrosinaseEC 1.14.18.1; LB24-AB; Monophenol monooxygenase; SK29-AB; Tumor rejection antigen ABHomo sapiens (human)
Catechol O-methyltransferaseEC 2.1.1.6Homo sapiens (human)
Aspartate aminotransferase, cytoplasmiccAspAT; EC 2.6.1.1; EC 2.6.1.3; Cysteine aminotransferase, cytoplasmic; Cysteine transaminase, cytoplasmic; cCAT; Glutamate oxaloacetate transaminase 1; Transaminase AHomo sapiens (human)
Phenylethanolamine N-methyltransferasePNMTase; EC 2.1.1.28; Noradrenaline N-methyltransferaseHomo sapiens (human)
Alcohol dehydrogenase 1AEC 1.1.1.1; Alcohol dehydrogenase subunit alphaHomo sapiens (human)
Dopamine beta-hydroxylaseEC 1.14.17.1; Dopamine beta-monooxygenaseHomo sapiens (human)
L-dopachrome tautomeraseDCT; DT; EC 5.3.3.12; L-dopachrome Delta-isomerase; Tyrosinase-related protein 2; TRP-2; TRP2Homo sapiens (human)
Amiloride-sensitive amine oxidase [copper-containing]DAO; Diamine oxidase; EC 1.4.3.22; Amiloride-binding protein 1; Amine oxidase copper domain-containing protein 1; Histaminase; Kidney amine oxidase; KAOHomo sapiens (human)
Aromatic-L-amino-acid decarboxylaseAADC; EC 4.1.1.28; DOPA decarboxylase; DDCHomo sapiens (human)
Aldehyde dehydrogenase, dimeric NADP-preferring EC 1.2.1.5; ALDHIII; Aldehyde dehydrogenase 3; Aldehyde dehydrogenase family 3 member A1Homo sapiens (human)
3-hydroxyanthranilate 3,4-dioxygenaseEC 1.13.11.6; 3-hydroxyanthranilate oxygenase; 3-HAO; h3HAO; 3-hydroxyanthranilic acid dioxygenase; HADHomo 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)

Compounds (46)

CompoundDescription
hydronium ion
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
glutamic acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
NADH
NorepinephrinePrecursor of epinephrine that is secreted by the ADRENAL MEDULLA and is a widespread central and autonomic neurotransmitter. Norepinephrine is the principal transmitter of most postganglionic sympathetic fibers, and of the diffuse projection system in the brain that arises from the LOCUS CERULEUS. It is also found in plants and is used pharmacologically as a sympathomimetic.
guaiacolAn agent thought to have disinfectant properties and used as an expectorant. (From Martindale, The Extra Pharmacopoeia, 30th ed, p747)
ascorbic acidA six carbon compound related to glucose. It is found naturally in citrus fruits and many vegetables. Ascorbic acid is an essential nutrient in human diets, and necessary to maintain connective tissue and bone. Its biologically active form, vitamin C, functions as a reducing agent and coenzyme in several metabolic pathways. Vitamin C is considered an antioxidant.
EpinephrineThe active sympathomimetic hormone from the ADRENAL MEDULLA. It stimulates both the alpha- and beta- adrenergic systems, causes systemic VASOCONSTRICTION and gastrointestinal relaxation, stimulates the HEART, and dilates BRONCHI and cerebral vessels. It is used in ASTHMA and CARDIAC FAILURE and to delay absorption of local ANESTHETICS.
levodopaThe naturally occurring form of DIHYDROXYPHENYLALANINE and the immediate precursor of DOPAMINE. Unlike dopamine itself, it can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to DOPAMINE. It is used for the treatment of PARKINSONIAN DISORDERS and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system.
tyrosineA non-essential amino acid. In animals it is synthesized from PHENYLALANINE. It is also the precursor of EPINEPHRINE; THYROID HORMONES; and melanin.
DopamineOne of the catecholamine NEUROTRANSMITTERS in the brain. It is derived from TYROSINE and is the precursor to NOREPINEPHRINE and EPINEPHRINE. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of receptors (RECEPTORS, DOPAMINE) mediate its action.
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.
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.
vanilmandelic acidA 3-O-methyl ether of 3,4-dihydroxymandelic acid. It is an end-stage metabolite of CATECHOLAMINES; EPINEPHRINE; and NOREPINEPHRINE.
dehydroerythorbic acidstructure given in first source
5,6-dihydroxy-2-indolylcarboxylic acid
normetanephrineA methylated metabolite of norepinephrine that is excreted in the urine and found in certain tissues. It is a marker for tumors.
4-hydroxyphenylacetic acid
leukodopachromeproposed metabolite in pathway from tyrosine to dopachrome; RN given refers to (S)-isomer; structure given in first source
3-methoxytyramineRN given refers to parent cpd
homovanillic acidA 3-O-methyl ETHER of (3,4-dihydroxyphenyl)acetic acid.
metanephrineProduct of epinephrine O-methylation. It is a commonly occurring, pharmacologically and physiologically inactive metabolite of epinephrine.
catecholRN given refers to unlabeled parent cpd
dopaquinoneproposed metabolite in pathway from tyrosine to dopachrome; RN given refers to cpd without isomeric designation; structure given in first source
alpha-ketoglutaric acid
fumarylacetoacetatemetabolite of tyrosine; RN given for (E)-isomer; structure in first source
3,4-dihydroxyphenylacetic acidA deaminated metabolite of LEVODOPA.
tyramineAn indirect sympathomimetic that occurs naturally in cheese and other foods. Tyramine does not directly activate adrenergic receptors, but it can serve as a substrate for adrenergic uptake systems and MONOAMINE OXIDASE to prolong the actions of adrenergic transmitters. It also provokes transmitter release from adrenergic terminals and may be a neurotransmitter in some invertebrate nervous systems.
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)
MelaninsInsoluble polymers of TYROSINE derivatives found in and causing darkness in skin (SKIN PIGMENTATION), hair, and feathers providing protection against SUNBURN induced by SUNLIGHT. CAROTENES contribute yellow and red coloration.
methylaminemonovalent cation in pyruvate kinase catalysis; RN given refers to parent cpd
homogentisic acidDihydroxyphenylacetic acid with hydroxyls at the 2 and 5 positions of the phenyl ring.
3,4-dihydroxyphenylglycolnoradrenaline metabolite in mouse brain; RN given refers to cpd without isomeric designation
acetoacetic acid
oxaloacetic acidA dicarboxylic acid ketone that is an important metabolic intermediate of the CITRIC ACID CYCLE. It can be converted to ASPARTIC ACID by ASPARTATE TRANSAMINASE.
methoxyhydroxyphenylglycolSynthesized from endogenous epinephrine and norepinephrine in vivo. It is found in brain, blood, CSF, and urine, where its concentrations are used to measure catecholamine turnover.
5,6-dihydroxyindole
3,4-dihydroxyphenylacetaldehyde
(4-hydroxy-3-methoxyphenyl)acetaldehydestructure given in first source
3,4-dihydroxyphenylglycolaldehyde
s-adenosylmethioninePhysiologic methyl radical donor involved in enzymatic transmethylation reactions and present in all living organisms. It possesses anti-inflammatory activity and has been used in treatment of chronic liver disease. (From Merck, 11th ed)
creolinfrom refined coal tar oils
3,4-dihydroxymandelic acidmetabolite of L-dopa; RN given refers to parent cpd without isomeric designation
4-hydroxyphenylpyruvic acidRN given refers to parent cpd