Determination of Branched-chainl-Amino Acids and Their Keto Analogs with Leucine Dehydrogenase
نویسندگان
چکیده
منابع مشابه
Enzymic determination of branched-chain amino acids.
Transamination of branched-chain amino acids to (alpha-oxoglutarate, catalyzed by leucine aminotransferase coupled to the glutamate dehydrogenase reaction, is used in an enzymic assay for determination of branched-chain amino acids in serum and tissue homogenates. The coefficients of variation of the method within-day and day-to-day are 2.4 and 6.5%, respectively. Analytical recovery of physiol...
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چکیده ندارد.
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Background Maple Syrup Urine Disease (MSUD) is a rare autosomal recessive metabolic error, characterized by Branched Chain α-Keto-acid Dehydrogenase Complex (BCKDC) deficiency. Mutations in 3 genes can lead to abnormal metabolism and accumulation of leucine, isoleucine, valine and corresponding keto-acids. MSUD affects 1 in 185,000 infants globally. Seizure is a common presentation among neonat...
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ژورنال
عنوان ژورنال: Agricultural and Biological Chemistry
سال: 1978
ISSN: 0002-1369
DOI: 10.1080/00021369.1978.10863275