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Branched chain amino acids, catabolism disorders

METABOLIC DISORDERS OF BRANCHED-CHAIN AMINO ACID CATABOLISM... [Pg.259]

The catabolism of leucine, valine, and isoleucine presents many analogies to fatty acid catabolism. Metabolic disorders of branched-chain amino acid catabolism include hypervalinemia, maple syrup urine disease, intermittent branched-chain ketonuria, isovaleric acidemia, and methylmalonic aciduria. [Pg.262]

The main function of vitamin B12 is thought to be in the metabolism of amino acids. Thus, B,2 is involved in the conversion of homocysteine to methionine and in the catabolism of some branched-chain amino acids. The neurological disorder that is usually associated with vitamin B12 deficiency is due to progressive demyelination of nervous tissue, thought to be owing to a build up of the vitamin Bj2 substrate, methylmalonyl CoA. This probably interferes with the formation of the myelin sheath. [Pg.42]

See other pages where Branched chain amino acids, catabolism disorders is mentioned: [Pg.341]    [Pg.391]    [Pg.328]    [Pg.40]    [Pg.2]    [Pg.272]   
See also in sourсe #XX -- [ Pg.259 , Pg.260 , Pg.261 ]



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Amino acid disorders

Amino acids branched

Amino acids catabolism

Amino acids chains

Branched chain

Branched chain amino acids, catabolism

Branched-chain acids

Branched-chain amino

Chain branching

Chain disorder

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