| 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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| 3-methylcrotonyl |
the radical of 3-methylcrotonic acid; the thioester formed with coenzyme A, 3-methylcrotonyl CoA, is an intermediate in the degradation of leucine.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| 3-methylcrotonyl CoA carboxylase deficiency |
an autosomal recessive aminoacidopathy due to deficiency of methylcrotonoyl-CoA carboxylase, characterized by increased urine levels of 3-methylcrotonylglycine, 3-methylcrotonic acid, and 3-hydroxyisovaleric acid and variable presentation of mental retardation, central nervous system dysfunction, and muscular atrophy. Written also β-methylcrotonyl CoA carboxylase deficiency.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| 3-methylcrotonylglycine |
a conjugate of 3-methylcrotonic acid and glycine, formed and excreted in excess in the urine when 3-methylcrotonoyl-CoA carboxylase activity is impaired.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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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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