Menkes syndrome is a disorder of
High-Yield Explanation
Menkes kinky hair syndromeEtiologyFeaturesPrognosisX-linked metabolic disturbance of copper metabolismMutation in copper-transpoing ATP7A geneMental retardationHypocupremiaDecreased circulating ceruloplasminChildren with this disease often die within 5 years because of dissecting aneurysms or cardiac rupture(Ref: Harrison's 18/e p604)