🍪Menkes kinky hair disease-
ATP 7A gene defect- XLR
Copper distribution to body from sites of absorption like intestines,kidney is faulty (so, Cu accumulates here, but is deficient in other tissues)
🍪Wilson's disease-
ATP 7B gene- AR
Copper management in liver is faulty, Cu linking with ceruloplasmin and biliary excretion of excess Cu are severely impaired. So, non protein bound free copper gets into circulation and damages tissues
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