HISTOCHEMICAL EVIDENCE FOR A POSSIBLE PRIMARY BIOCHEMICAL LESION IN MUSCULAR DYSTROPHY

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Abstract
The presence of phosphate esters of adenosine, uridine, guanosine and cytidine, together with DPN and TPN, in the proliferating endomysium of dystrophic muscle is of special significance since all except cytidine are related to the synthesis of sulphated''mucopolysaccharides. Acetyl amino sugars are activated as uridine nucleotides; uridine and guanosine phosphate esters play a part in the activation of sugars inpolysaccharide synthesis; adenosine and uridine nucleotides also play an essential role in sulphation of mucopolysaccharides; evidence supports the role of uridine nucleotides in chondroitin sulphate synthesis. DPN and TPN also mediate some mucopolysaccharide synthetic processes. All the above substances are involved in a cycle known as the "uridine nucleotide shunt." It is suggested that muscular dystrophy represents a congenital defect in this shunt in the connective tissue of dystrophic muscle. This theory and its implications are discussed.

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