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A branched-chain amino acid (BCAA) is an amino acid having an aliphatic side-chain with a branch (a central carbon atom bound to three or more carbon atoms). Among the proteinogenic amino acids, there are three BCAAs: leucine, isoleucine, and valine. [1] Non-proteinogenic BCAAs include 2-aminoisobutyric acid and alloisoleucine. Leucine ...
Due to the disturbed branched-chain amino acids (BCAA) metabolism, they are among the classical organic acidemias. [ 2 ] Methylmalonic acidemias have varying diagnoses, treatment requirements and prognoses, which are determined by the specific genetic mutation causing the inherited form of the disorder.
The branched-chain amino acids include isoleucine, leucine and valine. [1] Organic acids refer to the amino acids and certain odd-chained fatty acids which are affected by these disorders. The four main types of organic acidemia are: methylmalonic acidemia, propionic acidemia, isovaleric acidemia, and maple syrup urine disease. [1]
Low protein diets rapidly reduce fat and restores normal insulin sensitivity to diet-induced obese mice. [15] Specifically restricting consumption of the three branched-chain amino acids leucine, isoleucine and valine is sufficient to promote leanness and improve regulation of blood glucose. [16]
The blood concentration of leucine and isoleucine is measured relative to other amino acids to determine if the newborn has a high level of branched-chain amino acids. Once the newborn is 2–3 days old the blood concentration of branched-chain amino acids like leucine is greater than 1000 μmol/L and alternative screening methods are used.
The essential amino acids valine, methionine, isoleucine, and threonine can not be converted and this leads to a buildup of propionyl-CoA. Instead of being converted to methylmalonyl-CoA, propionyl-CoA is then converted into propionic acid, which builds up in the bloodstream. This in turn causes an accumulation of dangerous acids and toxins ...
In Corynebacterium glutamicum, BrnFE exports branched chain amino acids isoleucine, leucine, and valine, as well as methionine and homoserine. [3] [5] [8] The greatest induction of BrnFE expression occurs under high methionine concentrations, suggesting that methionine may actually be the native substrate rather than a branched-chain amino acid ...
Specifically, two essential branched-chain amino acids (leucine and isoleucine) are metabolized differently. [ citation needed ] Individuals lacking functional biotinidase enzymes can still have normal carboxylase activity if they ingest adequate amounts of biotin.