4,5-dihydroxyhexanoic acid (BioDeep_00000170062)

   

human metabolite


代谢物信息卡片


4,5-dihydroxyhexanoic acid

化学式: C6H12O4 (148.0736)
中文名称:
谱图信息: 最多检出来源 () 0%

分子结构信息

SMILES: CC(C(CCC(=O)O)O)O
InChI: InChI=1S/C6H12O4/c1-4(7)5(8)2-3-6(9)10/h4-5,7-8H,2-3H2,1H3,(H,9,10)

描述信息

4,5-dihydroxyhexanoic acid or DHHA is a water-soluble organic acid. It probably arises from the reaction of succinic semialdehyde with an intermediate in the pyruvate dehydrogenase pathway. DHHA is found in the urine and appears to be a marker for Succinic semialdehyde dehydrogenase (SSADH) deficiency (PMID: 3126356 ). SSADH is a disorder that arises from a defect in gamma-aminobutyric acid catabolism, resulting in the accumulation of gamma-hydroxybutyric acid (GHB), 2,4-dihydroxybutyric acid, adipic acid as well as elevated levels of 4,5-dihydroxyhexanoic acid, and 4,5-dihydroxyhexanoic acid lactone. SSADH causes neurological and cognitive disorders of varying severity (PMID: 3126356 ). Children with SSADH deficiency usually manifest with developmental delay, behavioral symptoms, language dysfunction, seizures, hypotonia, extrapyramidal symptoms, and ataxia (PMID: 32055132).

同义名列表

3 个代谢物同义名

4,5-dihydroxyhexanoic acid; 4,5-Dihydroxyhexanoate; DHHA



数据库引用编号

3 个数据库交叉引用编号

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代谢反应

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1 个相关的物种来源信息

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  • PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
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亚细胞结构定位 关联基因列表


文献列表

  • Eduard A Struys, Nanda M Verhoeven, Erwin E W Jansen, Herman J Ten Brink, Maneesh Gupta, Terry G Burlingame, Lawrence S Quang, Timothy Maher, Piero Rinaldo, O Carter Snead, Amy K Goodwin, Elise M Weerts, P Rand Brown, Tonya C Murphy, Mathew J Picklo, Cornelius Jakobs, K Michael Gibson. Metabolism of gamma-hydroxybutyrate to d-2-hydroxyglutarate in mammals: further evidence for d-2-hydroxyglutarate transhydrogenase. Metabolism: clinical and experimental. 2006 Mar; 55(3):353-8. doi: 10.1016/j.metabol.2005.09.009. [PMID: 16483879]
  • K M Gibson, D S M Schor, M Gupta, W S Guerand, H Senephansiri, T G Burlingame, H Bartels, B M Hogema, T Bottiglieri, W Froestl, O C Snead, M Grompe, C Jakobs. Focal neurometabolic alterations in mice deficient for succinate semialdehyde dehydrogenase. Journal of neurochemistry. 2002 Apr; 81(1):71-9. doi: 10.1046/j.1471-4159.2002.00784.x. [PMID: 12067239]