Amino Acid Metabolism — Flashcards

_____ (from tyrosine) may be converted to maleylacetoacetate via the enzyme homogentisate oxidase

In the liver, alanine transfers its NH3 group to _____, forming glutamate and pyruvate

_____ is eventually broken down into acetyl-CoA via the enzyme branched-chain α-ketoacid dehydrogenase

The urea cycle begins with _____, CO2, and 2 ATP

_____ and valine are eventually broken down into propionyl-CoA via the enzyme branched-chain alpha-ketoacid dehydrogenase

_____ may be converted to cystathionine via the enzyme cystathionine synthase (with B6 as a cofactor)

Type _____ maple syrup urine disease (MSUD) results from the mutation in the genes encoding dihydrolipoyl transacylase.

What enzyme deficiencies may cause homocystinuria? _____ and methionine synthase deficiencies

_____ disease is due to a deficiency of neutral amino acid transporters (especially tryptophan) in the proximal renal tubular cells and enterocytes

_____ is converted to dihydroxyphenylalanine (DOPA) via the enzyme tyrosine hydroxylase (with tetrahydrobiopterin as a cofactor)