79607-8
Saccharopine [Moles/volume] in Serum or Plasma
Active
Part Description
LP201690-7 Saccharopine
Saccharopine is an intermediate in the degradation of lysine, formed by condensation of lysine and alpha-ketoglutarate. The saccharopine pathway is the main route for lysine degradation in mammal and its first two reactions are catalyzed by enzymatic activities known as lysine-oxoglutarate reductase (LOR) and saccharopine dehydrogenase (SDH), which reside on a single bifunctional polypeptide (EC EC 1.5.1.8, LOR/SDH). The reactions involved by saccharopine dehydrogenases have a very strict substrate specificity for L-lysine, 2-oxoglutarate and NADPH. LOR/SDH has been detected in a number of mammalian tissues, mainly in the liver and kidney, contributing not only to the general nitrogen balance in the organism but also to the controlled conversion of lysine into ketone bodies. A tetrameric form has also been observed in human liver and placenta. LOR activity has also been detected in brain mitochondria during embryonic development, and this opens the question of whether the degradation of lysine has any functional significance during brain development and puts a new focus on the nutritional requirements for lysine in gestation and infancy. Finally, LOR and/or SDH deficiencies seem to be involved in a human autosomic genetic disorder known as familial hyperlysinemia, which is characterized by serious defects in the functioning of the nervous system, and characterized by deficiency in lysine-ketoglutarate reductase, saccharopine dehydrogenase, and saccharopine oxidoreductase activities. Saccharopinuria (high amounts of saccharopine in the urine) and saccharopinemia (an excess of saccharopine in the blood) are conditions present in some inherited disorders of lysine degradation. PMID: 463877 PMID: 10567240 PMID: 10772957 PMID: 4809305
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,
Fully-Specified Name
- Component
- Saccharopine
- Property
- SCnc
- Time
- Pt
- System
- Ser/Plas
- Scale
- Qn
- Method
Additional Names
- Short Name
- Saccharopine SerPl-sCnc
- Display Name
- Saccharopine [Moles/Vol]
- Consumer Name Alpha Get Info
- Saccharopine, Blood
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.56
- Change Reason
- Changed Class from CHEM.ESOTERIC to CHEM and added Esoteric tag for more flexibility
- Order vs. Observation
- Observation
Member of these Panels
LOINC | Long Common Name |
---|---|
35083-5 | Amino acids panel [Moles/volume] - Serum or Plasma |
Language Variants Get Info
Tag | Language | Translation |
---|---|---|
de-DE | German (Germany) | Saccharopin: |
es-MX | Spanish (Mexico) | Sacaropina: |
es-ES | Spanish (Spain) | Sacaropina: Synonyms: Cuantitativo |
fr-FR | French (France) | Saccharopine: |
it-IT | Italian (Italy) | Saccaropina: Synonyms: Chimica Concentrazione di Sostanza Plasma Punto nel tempo (episodio) Siero Siero o Plasma |
nl-NL | Dutch (Netherlands) | saccharopine: |
tr-TR | Turkish (Turkey) | Sakkaropin: |
zh-CN | Chinese (China) | 酵母氨酸: Synonyms: 克分子浓度; |
Example Units
Unit | Source |
---|---|
umol/L | Example UCUM Units |
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