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A type of muscular dystrophy, called severe childhood autosomal recessive muscular dystrophy (SCARMD), results from a mutation in the gene for a 50-kD muscle protein. The defective protein leads to muscle necrosis. Detailed studies of this protein have revealed that an arginine residue at position 98 has been mutated to a histadine. Why might replacing an arginine with a histadine result in a defective protein?

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