LP18605-3
Pyruvate dehydrogenase
Active
Description
Pyruvate dehydrogenase (E1) is the first component enzyme of pyruvate dehydrogenase complex (PDC). The pyruvate dehydrogenase complex contributes to transforming pyruvate into acetyl-CoA by a process called pyruvate decarboxylation. Acetyl-CoA may then be used in the citric acid cycle to carry out cellular respiration, so pyruvate dehydrogenase contributes to linking the glycolysis metabolic pathway to the citric acid cycle and releasing energy via NADH. EC 1.2.4.1.
Pyruvate dehydrogenase is an autoantigen recognized in primary biliary cirrhosis, a form of acute liver failure. These antibodies appear to recognize oxidized protein that has resulted from inflammatory immune responses. Some of these inflammatory responses are explained by gluten sensitivity.
Pyruvate dehydrogenase (PDH) deficiency is a congenital degenerative metabolic disease resulting from a mutation of the pyruvate dehydrogenase complex (PDC) located on the X chromosome. Although defects have been identified in all 3 enzymes of the complex, the E1-α subunit is predominantly the culprit. Malfunction of the citric acid cycle due to PDH deficiency deprives the body of energy and leads to an abnormal buildup of lactate. PDH deficiency is a common cause of lactic acidosis in newborns and often presents with severe lethargy, poor feeding, tachypnea, and cases of death have occurred.
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Source: Wikipedia
, Pyruvate dehydrogenase
Basic Part Properties
- Part Name
- Pyruvate dehydrogenase
- Part Display Name
- Pyruvate dehydrogenase
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2000-05-04
- Construct for LOINC Short Name
- PDH
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Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| zh-CN | Chinese (China) | 丙酮酸脱氢酶 Synonyms: PDH; |
| tr-TR | Turkish (Turkey) | Piruvat dehidrojenaz |
| it-IT | Italian (Italy) | Piruvato deidrogenasi |
| es-ES | Spanish (Spain) | Piruvato deshidrogenasa |
| ru-RU | Russian (Russian Federation) | Пируват дегидрогеназа Synonyms: Пируватдегидрогеназа |
| nl-NL | Dutch (Netherlands) | pyruvaatdehydrogenase |
| el-GR | Greek (Greece) | Πυροσταφυλική αφυδρογονάση Synonyms: Πυροσταφυλική αφυδρογονάση |
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