(A) Patient can not be treated by dietary
regulation
(B) Without treatment death, of patient may occur
by the end of second year of life
(C) Blood levels of leucine, isoleucine and serine
are increased
(D) Excessive brain damage
Tag: Biochemistry Mcqs
Alkaptonuria occurs due to deficiency of the enzyme:
(A) Maleylacetoacetate isomerase
(B) Homogentisate oxidase
(C) p-Hydroxyphenylpyruvate hydroxylase
(D) Fumarylacetoacetate hydrolase
Maple syrup urine disease becomes evident in extra uterine life by the end of?
(A) First week
(B) Second week
(C) Third week
(D) Fourth week
An inborn error, maple syrup urine disease is due to deficiency of the enzyme:
(A) Isovaleryl-CoAhydrogenase
(B) Phenylalnine hydroxylase
(C) Adenosyl transferase
(D) α-Ketoacid decarboxylase
In glycinuria daily urinary excretion of glycine ranges from?
(A) 100–200 mg
(B) 300–500 mg
(C) 600–1000 mg
(D) 1100–1400 mg
Increased urinary indole acetic acid is diagnostic of?
(A) Maple syrup urine disease
(B) Hartnup disease
(C) Homocystinuia
(D) Phenylketonuria
An important finding in glycinuria is?
(A) Excess excretion of oxalate in the urine
(B) Deficiency of enzyme glycinase
(C) Significantly increased serum glycine level
(D) Defect in renal tubular reabsorption of glycine
An important finding in Histidinemia is?
(A) Impairment of conversion of α-Glutamate to
α-ketoglutarate
(B) Speech defect
(C) Decreased urinary histidine level
(D) Patients can not be treated by diet