<prompt> Menkes Kinky hair syndrome is characterized by congenital deficiency ofSerum ceruloplasminSerum copperFerochelataseCopper binding ATPase  <response>: Ans. d Copper binding ATPase Ref. Harper 28th573-4; H-17th449.Menkes disease kinky or steely hair disease is a disorder of copper metabolism that occurs due toMutations in the Gene Encoding a Copper-Binding P-Type ATPase.MENKES DISEASE It is X-linked, affects only male infants, involves the nervous system, connective tissue, and vasculature, and is usually fatal in infancy. When altered by mutation, copper is not mobilized normally from the intestine, in which it accumulates, as it does in a variety of other cells and tissues, from which it cannot exit. Despite the accumulation of copper, the activities of many copper-dependent enzymes are decreased, perhaps be- cause of a defect of its incorporation into the apoenzymes. Normal liver expresses very little of ATPase, which explains the absence of hepatic involvement in Menkes disease.Wilson Disease Is Also Due to Mutations in a Gene Encoding a Copper-Binding P-Type ATPase. 