Selective Amino Acid Metabolism Part II Flashcards
1
Q
What deficiency causes classic homocysteinemia?
A
defect in cystathionine B synthase
2
Q
What causes non-classic homocysteinemia?
A
defect in methionine synthase, methyl-colbalamin or folate and its derivatives
3
Q
What direction is the lens dislocation in homocysteinuria?
A
inferonasal
4
Q
What accumulates in classical homocystenemia
A
homocysteine and methionine
5
Q
What accumulates in non-classical homocystenemia?
A
only homocysteine
