Defect in Menke disease:
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.
Lysyl hydroxylase
Lysyl oxidase
Prolyl hydroxylase
Prolyl oxidase
NEET 2019