Which amino acids accumulate in maple syrup urine disease?
AValine
BLeucine
CIsoleucine
DAll branched-chain amino acids
A young boy with a thin build and long fingers presents with diminished vision. On examination, subluxation of the lens is observed, and cystathionine synthase deficiency is detected. Which amino acid should the patient be supplemented with?
ATyrosine
BSerine
CCysteine
DMethionine
EGlycine
An infant is brought by his parents with complaints that his urine turns black on standing. Which of the following metabolic disorders is likely?
APhenylketonuria
BAlkaptonuria
CHomocystinuria
DMaple syrup urine disease
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