Branched chain amino acid compounds and their corresponding alpha keto acids
and the enzymatic defect in MSUD was demonstrated by Dancis et al.
lactate, urates, keto acids
and others, enabling the preservation of complex and efficient cell functions related to acid-base balance (12-14,17).
Catabolism of these amino acids is initiated by transamination reaction with alpha-ketoglutarate to form glutamate and branched-chain keto acids
The quantitation of a- keto acids
by fluorescence HPLC has been reviewed .
Rexim's 17,000m2 Ham site mainly produces high quality pharmaceutical grades of amino acids, their salts and derivatives, as well as peptides and keto acids
Accumulation of these 3 amino acids and their corresponding keto acids
leads to encephalopathy and progressive neurodegeneration in untreated infants.
Increased amounts of keto acids
, including phenylpyruvate, were detected during analysis of the urine.
The amination of keto acids
is assumed to be more important then the elimination of ammonia (Fairbairn, 1957).
Poor preservation of samples will lead to nonenzymatic conversion of all keto acids
to the respective hydroxyacid; for example, acetoacetate is converted to 3-hydroxybutyrate, and 2-ketoglutarate is converted to 2-hydroxyglutarate.
The reagent blank was prepared following the same procedure, without the addition of a- keto acids
During this reaction alpha keto acids
converted into amino acids may be utilized on the protein synthesis, thus regulating the carbohydrate and protein metabolisms (Knox and Greengard, 1965; Martin et al 1981).
Influence of branched-chain amino acids and branched-chain keto acids
on protein synthesis in isolated hepatocytes.