Discussion
One of the common symptoms of a metabolic disorder is that the patients have lighter hair and skin colour than their siblings. What is the name of this disorder ?
Answer: C
The disorder is Phenylketonuria.
In phenylketonuria the enzyme phenylalanine hydroxylase is deficient, so phenylalanine is not converted to tyrosine. Tyrosine is the starting material for melanin, and phenylalanine also inhibits melanin synthesis, so untreated patients often have fairer skin and hair than their siblings, along with intellectual disability.
Alkaptonuria causes urine that darkens on standing and dark pigment in cartilage. Tay-Sachs disease is a ganglioside storage disorder affecting the nervous system, and Cori's disease is a glycogen storage disease caused by deficiency of the debranching enzyme.
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