Defect in Menke disease:: (A) Lysyl hydroxylase, (B) Lysyl oxidase, (C) Prolyl hydroxylase, (D) Prolyl oxidase
Answer is B. MENKE DISEASE / kinky OR STEEL HAIR SYNDROME- deficiency of copper results in defective crosslinking of collagen and elastin by the copper-dependent enzyme lysyl oxidase. Defect in dietary absorption of copper Die with in 5 years of age Kinky hair and growth retardation. menke disease is X linked disease due to mutation in ATP 7 A gene. This gene is present on Xp 12-13.