Full 2L QBank
Biochemistry Structure and function of protein 818c2ac8

An infant presented to the OPD with a history of vomiting and poor feeding. Musty odor is present in the baby's sweat and urine. Guthrie test was done and it was found to be positive. All are true regarding this disease except:

A
Phenylacetate positive in urine
B
Tandem mass spectrometry is the gold standard test
C
Phenylalanine hydroxylase enzyme defect
D
mental retardation is absent
High-Yield Explanation
The given clinical condition is suggestive of phenylketonuria (PKU) PKU is caused by the deficiency of an enzyme phenylalanine hydroxylase. Guthrie bacterial inhibition test is a screening test for phenylketonuria. This test uses a phenylalanine requiring strain of Bacillus subtilis. Ferric chloride test is also used as a screening test for PKU. Tandem mass spectrometry is the gold standard investigation Reference: Harpers illustrated biochemistry 30th edition

Related Biochemistry MCQs

Practice 2,00,000+ NEET PG Questions Free

Timed mock tests, mistake queue analytics, audio lectures & zero attempt limits on i❤️Exams.

Start Free Mock Test Now