<prompt> Defect in Menke disease:Lysyl hydroxylaseLysyl oxidaseProlyl hydroxylaseProlyl oxidase  <response>: 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. 