Content of methylhistidines in normal and pathological human skeletal muscles

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Abstract
The content of 3‐methylhistidine (3‐MH) and 1‐methylhistidine (1‐MH) was measured in muscle biopsy specimens from 13 normal controls, 19 patients with Duchenne muscular dystrophy, 8 limb‐girdle disease patients, and 23 disease controls with different forms of muscular pathology. 3‐MH and 1‐MH concentrations in normal human muscle did not appear to be influenced by sex, body weight, and age, at least for subjects in the 10–60 year age group examined. Skeletal muscle 1‐MH levels did not significantly differ from mean control values in any of the pathologies investigated. In the patient population examined, the mean 3‐MH level per unit of noncollagen protein (NCP) was significantly lower than normal in Duchenne dystrophy only, the reduction being related to disease severity. The significantly lower concentrations of 3‐MH in muscle of Duchenne patients indicate the importance of measuring 3‐MH in diseased muscle to obtain reliable estimates of the myofibrillar protein catabolic rate.