Which amino acids accumulate in maple syrup urine disease?
AValine
BLeucine
CIsoleucine
DAll branched-chain amino acids
Because free ammonia in the blood is toxic to the body, it is transported in which of the following non-toxic forms?
AGlutamine and urea
BAlanine and glutamine
CPhenylalanine and methionine
DHistidine and urea
An infant presented with vomiting, malnutrition, blue eyes, blonde hair & fair skin. On investigation, Guthrie test was positive. All are true regarding this disease EXCEPT:
APhenyl acetate positive in urine
BMental retardation is present
CHypopigmentation due to tryptophan deficiency
DDue to PAH enzyme defect
+ 7 more in the PDF
Browse all chapters