"Menke's disease" is a disease of-

Menkes kinky hair syndrome is an X-linked metabolic disturbance of copper metabolism characterized by mental retardation, hypocupremia, and decreased circulating ceruloplasmin. This syndrome is caused by mutations in the copper-transpoing ATP7A gene. Children with this disease often die within 5 years because of dissecting aneurysms or cardiac rupture . Ref - Harrisons 20e p2318