LP18408-2
2-Oxoadipate
Active
Descriptions
2-Oxoadipic acid is produced from lysine in the cytosol of cells via the saccharopine and the pipecolic acid pathways. Catabolites of hydroxylysine and tryptophan enter these pathways as 2-aminoadipic- -semialdehyde and 2-oxoadipate, respectively. In the matrix of mitochondria, 2-oxoadipate is decarboxylated to glutaryl-CoA by the 2-oxoadipate dehydrogenase complex and then converted to acetyl-CoA. 2-Oxoadipic aciduria is an in-born error of metabolism of lysine, tryptophan, and hydroxylysine, in which abnormal quantities of 2-aminoadipic acid are found in body fluids along with 2-oxoadipic acid. Patients with 2-Oxoadipic acidemias are mentally retarded with hypotonia or seizures. 2-Oxoadipic Aciduria can occur in patients with Kearns-Sayre Syndrome, a progressive disorder with onset prior to 20 years of age in which multiple organ systems are affected, including progressive external ophthalmoplegia, retinopathy, and the age of onset, and these are associated classically with abnormalities in cardiac conduction, cerebellar signs, and elevated cerebrospinal fluid protein. PMID: 10655159, PMID: 16183823, PMID: 11083877 Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.) Source: Human Metabolome Database
α-Ketoadipic acid (or 2-oxoadipate) is an intermediate in the metabolism of lysine. Copyright Text is available under the Creative Commons Attribution/Share-Alike License. See http://creativecommons.org/licenses/by-sa/3.0/ for details. Source: Wikipedia, Wikipedia
Basic Part Properties
- Part Display Name
- 2-Oxoadipate
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2000-05-04
- Construct for LOINC Short Name
- 2-Oxoadipate
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Language Variants Get Info
Tag | Language | Translation |
---|---|---|
zh-CN | Chinese (China) | 2-氧代己二酸 Synonyms: 2Oxo-己二酸 |
fr-CA | French (Canada) | 2-oxoadipate |
et-EE | Estonian (Estonia) | 2-oksoadipaat |
es-ES | Spanish (Spain) | 2-Oxoadipato |
it-IT | Italian (Italy) | 2-Ossoadipato |
tr-TR | Turkish (Turkey) | 2-Okzoadipat |
ru-RU | Russian (Russian Federation) | 2-Оксоадипат |
nl-NL | Dutch (Netherlands) | 2-oxoadipaat |
fr-BE | French (Belgium) | 2-oxoadipate |
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