WordNet
- an acid formed as an intermediate product of the metabolism of tyrosine and phenylalanine (同)alcapton, homogentisic_acid
- a rare recessive metabolic anomaly marked by ochronosis and the presence of alkapton in the urine (同)alcaptonuria
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/14 10:44:36」(JST)
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Homogentisic acid
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| Names |
| IUPAC name
(2,5-Dihydroxyphenyl)acetic acid
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| Other names
Melanic acid
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| Identifiers |
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CAS Registry Number
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451-13-8 Y |
| ChEBI |
CHEBI:44747 Y |
| ChemSpider |
759 Y |
| DrugBank |
DB08327 Y |
InChI
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InChI=1S/C8H8O4/c9-6-1-2-7(10)5(3-6)4-8(11)12/h1-3,9-10H,4H2,(H,11,12) Y
Key: IGMNYECMUMZDDF-UHFFFAOYSA-N Y
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InChI=1/C8H8O4/c9-6-1-2-7(10)5(3-6)4-8(11)12/h1-3,9-10H,4H2,(H,11,12)
Key: IGMNYECMUMZDDF-UHFFFAOYAK
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| Jmol-3D images |
Image |
| KEGG |
C00544 Y |
| MeSH |
Homogentisic+acid |
| PubChem |
780 |
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| Properties |
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Molecular formula
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C8H8O4 |
| Molar mass |
168.15 g·mol−1 |
| Melting point |
150 °C (302 °F; 423 K) |
Except where noted otherwise, data is given for materials in their standard state (at 25 °C (77 °F), 100 kPa)
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| Y verify (what is: Y/N?) |
| Infobox references |
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Homogentisic acid (2,5-dihydroxyphenylacetic acid) is a phenolic acid found in Arbutus unedo (strawberry-tree) honey.[1] It is also present in the bacterial plant pathogen Xanthomonas campestris pv. phaseoli[2] as well as in the yeast Yarrowia lipolytica[3] where it is associated with the production of brown pigments.
It is less commonly known as melanic acid, the name chosen by William Prout.
Human pathology
Accumulation of excess homogentisic acid and its oxide, named alkapton, is a result of the failure of the enzyme homogentisic acid 1,2-dioxygenase (typically due to a mutation) in the degradative pathway of tyrosine, consequently associated with alkaptonuria.[4]
Intermediate
It is an intermediate in the catabolism of aromatic amino acids such as phenylalanine and tyrosine.
References
- ^ Paolo Cabras, Alberto Angioni, Carlo Tuberoso, Ignazio Floris, Fabiano Reniero, Claude Guillou and Stefano Ghelli (1999). "Homogentisic Acid: A Phenolic Acid as a Marker of Strawberry-Tree (Arbutus unedo) Honey". J. Agric. Food Chem. 47 (10): 4064–4067. doi:10.1021/jf990141o.
- ^ Goodwin PH and Sopher CR (1994). "Brown pigmentation of Xanthomonas campestris pv. phaseoli associated with homogentisic acid". Canadian Journal of Microbiology 40 (1): 28–34. doi:10.1139/m94-005.
- ^ Alexandra Carreira, Luísa M. Ferreira and Virgílio Loureiro (2001). "Brown Pigments Produced by Yarrowia lipolytica Result from Extracellular Accumulation of Homogentisic Acid". Appl Environ Microbiol 67 (8): 3463–3468. doi:10.1128/AEM.67.8.3463-3468.2001.
- ^ Phornphutkul C, Introne WJ, Perry MB et al. (2002). "Natural history of alkaptonuria". New England Journal Medicine 347 (26): 2111–21. doi:10.1056/NEJMoa021736. PMID 12501223.
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Amino acid metabolism metabolic intermediates
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| K→acetyl-CoA |
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lysine→
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- Saccharopine
- Allysine
- α-Aminoadipic acid
- α-Ketoadipate
- Glutaryl-CoA
- Glutaconyl-CoA
- Crotonyl-CoA
- β-Hydroxybutyryl-CoA
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leucine→
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- α-Ketoisocaproic acid
- Isovaleryl-CoA
- 3-Methylcrotonyl-CoA
- 3-Methylglutaconyl-CoA
- HMG-CoA
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|
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tryptophan→alanine→
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- N'-Formylkynurenine
- Kynurenine
- Anthranilic acid
- 3-Hydroxykynurenine
- 3-Hydroxyanthranilic acid
- 2-Amino-3-carboxymuconic semialdehyde
- 2-Aminomuconic semialdehyde
- 2-Aminomuconic acid
- Glutaryl-CoA
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|
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| G |
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G→pyruvate→citrate
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glycine→serine→
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- glycine→creatine: Glycocyamine
- Phosphocreatine
- Creatinine
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G→glutamate→
α-ketoglutarate
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histidine→
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- Urocanic acid
- Imidazol-4-one-5-propionic acid
- Formiminoglutamic acid
- Glutamate-1-semialdehyde
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proline→
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- 1-Pyrroline-5-carboxylic acid
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arginine→
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- Agmatine
- Ornithine
- Cadaverine
- Putrescine
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other
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- cysteine+glutamate→glutathione: γ-Glutamylcysteine
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G→propionyl-CoA→
succinyl-CoA
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valine→
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- α-Ketoisovaleric acid
- Isobutyryl-CoA
- Methacrylyl-CoA
- 3-Hydroxyisobutyryl-CoA
- 3-Hydroxyisobutyric acid
- 2-Methyl-3-oxopropanoic acid
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isoleucine→
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- 2,3-Dihydroxy-3-methylpentanoic acid
- 2-Methylbutyryl-CoA
- Tiglyl-CoA
- 2-Methylacetoacetyl-CoA
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methionine→
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- generation of homocysteine: S-Adenosyl methionine
- S-Adenosyl-L-homocysteine
- Homocysteine
- conversion to cysteine: Cystathionine
- alpha-Ketobutyric acid+Cysteine
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threonine→
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propionyl-CoA→
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G→fumarate
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phenylalanine→tyrosine→
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- 4-Hydroxyphenylpyruvic acid
- Homogentisic acid
- 4-Maleylacetoacetic acid
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G→oxaloacetate
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| Other |
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Index of inborn errors of metabolism
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| Description |
- Metabolism
- Enzymes and pathways: citric acid cycle
- pentose phosphate
- glycoproteins
- glycosaminoglycans
- phospholipid
- cholesterol and steroid
- sphingolipids
- eicosanoids
- amino acid
- urea cycle
- nucleotide
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| Disorders |
- Citric acid cycle and electron transport chain
- Glycoprotein
- Proteoglycan
- Fatty-acid
- Phospholipid
- Cholesterol and steroid
- Eicosanoid
- Amino acid
- Purine-pyrimidine
- Heme metabolism
- Symptoms and signs
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| Treatment |
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Index of biochemical families
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| Carbohydrates |
- Alcohols
- Glycoproteins
- Glycosides
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| Lipids |
- Eicosanoids
- Fatty acids
- Glycerides
- Phospholipids
- Sphingolipids
- Steroids
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| Nucleic acids |
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| Proteins |
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| Other |
- Tetrapyrroles
- intermediates
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Phenolic acids (C6-C1) and their glycosides
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| Monohydroxybenzoic acids |
- 3-Hydroxybenzoic acid
- 4-Hydroxybenzoic acid
- Salicylic acid (2-Hydroxybenzoic acid)
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Glycosides
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- p-Hydroxybenzoic acid glucoside
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Acetylated
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- Methylparaben (Methyl p-hydroxybenzoic acid)
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| Dihydroxybenzoic acids |
- 2,3-Dihydroxybenzoic acid (Hypogallic acid)
- 2,4-Dihydroxybenzoic acid
- 2,5-Dihydroxybenzoic acid (Gentisic acid)
- 2,6-Dihydroxybenzoic acid
- 3,4-Dihydroxybenzoic acid (Protocatechuic acid)
- 3,5-Dihydroxybenzoic acid
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Acetylated
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- Ethyl protocatechuate
- Orsellinic acid
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| Trihydroxybenzoic acids |
- Gallic acid
- Phloroglucinol carboxylic acid
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Glycosides
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- Bergenin
- Norbergenin
- Theogallin
- Chebulic acid
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Acetylated
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- Ethyl gallate
- Eudesmic acid
- Methyl gallate
- Syringic acid
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English Journal
- Nine cases of Alkaptonuria in one family in southern Jordan.
- Al-Sbou M, Mwafi N.SourceDepartment of Pharmacology, Alkptonuria Research Office, Faculty of Medicine, Mutah University, P.O. Box 7, Mutah, Karak 61710, Jordan. malsbou@mutah.edu.jo
- Rheumatology international.Rheumatol Int.2012 Mar;32(3):621-5. Epub 2010 Dec 3.
- Alkaptonuria is a rare autosomal recessive metabolic disorder characterized by a deficiency of homogentisate 1,2-dioxygenase (HGO) in the liver. This results in excretion of large quantities of homogentisic acid (HGA) (also called alkapton) in the urine and a slowly progressive deposition of homogen
- PMID 21127875
- [Formation of the urinary stones in alkaptonuria patients].
- Zadina J, Průsa R, Goliás V, Staifová K.SourceUstav klinické biochemie a patobiochemie 2. LF UK a FNM, Praha. jiri.zadina@fnmotol.cz
- Casopís lékar̆ů c̆eských.Cas Lek Cesk.2008;147(6):330-2.
- Alkaptonuria is an autosomal recessive disease with late complications (arthritis and ochronosis). Excretion of alkapton (homogentisic acid) in the urine is due to congenital lack of the enzyme homogentisate 1,2-dioxygenase, which mediates the essential step in the catabolism of phenylalanine and ty
- PMID 18724531
Japanese Journal
- 尿検査 アルカプトン(ホモゲンチジン酸) (広範囲 血液・尿化学検査,免疫学的検査(第7版・1)その数値をどう読むか) -- (一般検査(尿・髄液・糞便検査を含む))
- Alkapton尿症に合併した腰部脊椎管狭窄症の1症例 (第59回中部日本整形外科災害外科学会) -- (腰部脊椎管狭窄)
Related Links
- Alkapton definition, homogentisic acid. See more. Thesaurus Translate Reference Word of the Day Blog Slideshows Apps by Dictionary My Account Log Out Log In follow Dictionary.com Dictionary Thesaurus Translate More...
- al·kap·ton or al·cap·ton (ăl-kăp′tŏn′, -tən) n. See homogentisic acid. alkapton [al-kap´ton] a class of substances with an affinity for alkali, sometimes found in the urine and causing the condition known as alkaptonuria. The urinary ...
Related Pictures
★リンクテーブル★
[★]
- 関
- alcaptonuria、alkaptonuria
[★]
アルカプトン尿性関節症