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The class 3 defect results in an LDL receptor that is incapable of binding LDL

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There are four members of mitochondrial ABC subfamily B (ABCB6, ABCB7, ABCB8, and ABCB10), of which one, ABCB7, is located in the inner membrane of mitochondria and plays an essential role in cytosolic Fe-S cluster biogenesis and cellular iron homeostasis 6,7

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