| methylmalonicaciduria |
1. excess of methylmalonic acid in the urine. 2. methylmalonicacidemia.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| methylmalonyl |
the radical of methylmalonic acid; the thioester it forms with coenzyme A, methylmalonyl CoA, is an intermediate in the catabolism of certain amino acids and odd-number chain-length fatty acids.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| methylmalonyl-CoA epimerase |
[EC 5.1.99.1] an enzyme of the isomerase class that catalyzes the equilibration of the D- and L- isomers of methylmalonyl CoA. The reaction is part of the route by which three-carbon compounds from some amino acids and from odd number chain length fatty acids are used as fuels. Called also methylmalonyl-CoA racemase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| methylmalonyl-CoA mutase |
[EC 5.4.99.2] an enzyme of the isomerase class that catalyzes the isomerization of L- methylmalonyl coenzyme A to succinyl coenzyme A, requiring adenosylcobalamin as a coenzyme. The reaction is a step in the use of isoleucine, threonine, valine, propionate, and other odd number chain length fatty acids as fuels. Deficiency of enzyme activity, which may be caused by defects in the apoenzyme, in the coenzyme, or in cobalamin metabolism, results in methylmalonicacidemia.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| methylmalonyl-CoA racemase |
methylmalonyl-CoA epimerase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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