cystathioninuria A genetic disease affecting the metabolism of the amino acid methionine and its conversion to cysteine. May result in mental retardation if untreated. Treatment is by feeding a diet low in methionine and supplemented with cysteine, or, in some cases, by administration of high intakes of vitamin B6 (about 100–500 times the normal requirement).
More From encyclopedia.com
Phenylketonuria , Phenylketonuria (PKU) is an inherited disorder in which an enzyme (usually phenylalanine hydroxylase) crucial to the appropriate processing of the am… Maple Syrup Urine Disease , Maple Syrup Urine Disease Maple syrup urine disease (MSUD ), which is also known as branched-chain ketoaciduria, branched-chain alpha-keto acid dehyd… Tryptophan , tryptophan tryptophan (trĬp´təfăn), organic compound, one of the 20 amino acids commonly found in animal proteins. Only the l-stereoisomer appears in… Carnitine , Carnitine Description Carnitine is an amino acid that is essential for babies and nonessential for others. In this context, essential means it must b… galactosaemia , galactosaemia Genetic disease; inability to metabolize the sugar galactose. Unless galactose is excluded from the diet, the infants suffer mental ret… Phenylalanine , phenylalanine (fĕn´əlăl´ənēn´), organic compound, one of the 22 α-amino acids commonly found in animal proteins. Only the l-stereoisomer appears in m…
About this article
Updated About encyclopedia.com content Print Article
You Might Also Like