| 3-methylcrotonyl-CoA | (CH3)2C==CHCOSCoA;an intermediate in the degradation of l-leucine; accumulates in a deficiency of 3-methylcrotonyl-CoA carboxylase. 3-methylcrotonyl-CoA carboxylase, an enzyme in the pathway of l-leucine degradation that catalyses the reaction of 3-methylcrotonyl-CoA with CO2, ATP, and water to form ADP, orthophosphate, and 3-methylglutaconyl-CoA; a deficiency of this enzyme causes episodes of severe metabolic acidosis. (05 Mar 2000) |
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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