LP18437-1
Glutarate
Active
Descriptions
Glutaric acid (IUPAC systematic name:pentanedioic acid) is a simple five-carbon linear dicarboxylic acid. 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, Glutarate (Wikipedia)
Glutaric acid (glutarate) is a simple five-carbon linear dicarboxylic acid. The accumulation of glutaric acid ranging from slightly or intermittently elevated urinary glutaric acid to gross organic aciduria occurs in Glutaric aciduria. Glutaric aciduria type 1 is an autosomal-recessive disorder resulting from a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH) which is involved in the metabolism of lysine, hydroxylysine, and tryptophan. Glutaric aciduria type I lead to nonspecific developmental delay, hypotonia, and macrocephaly with cerebral atrophy of prenatal onset. Treatment is mainly based on restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3-hydroxyglutaric acid by restriction of natural protein in general and of lysine in particular. PMID: 17465389, PMID: 15505398 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
Basic Part Properties
- Part Display Name
- Glutarate
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2000-05-04
- Construct for LOINC Short Name
- Glutarate
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Language Variants Get Info
Tag | Language | Translation |
---|---|---|
zh-CN | Chinese (China) | 戊二酸 Synonyms: 戊二酸根; |
fr-CA | French (Canada) | Glutarate |
et-EE | Estonian (Estonia) | Glutaraat |
es-ES | Spanish (Spain) | Glutarato |
it-IT | Italian (Italy) | Glutarato |
tr-TR | Turkish (Turkey) | glutarat |
ru-RU | Russian (Russian Federation) | Глутарат |
nl-NL | Dutch (Netherlands) | glutaraat |
fr-BE | French (Belgium) | Glutarate |
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