Content of methylhistidines in normal and pathological human skeletal muscles.
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.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006639:Histidine / Q000031:analogs & derivatives*; D006801:Humans; D007223:Infant; D008297:Male; D008762:Methylhistidines / Q000378:metabolism*; D008875:Middle Aged; D009124:Muscle Proteins / Q000378:metabolism; D009132:Muscles / Q000378:metabolism*; D009133:Muscular Atrophy / Q000378:metabolism; D009135:Muscular Diseases / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000378:metabolism
D008762:Methylhistidines; D009124:Muscle Proteins; C028120:1-methylhistidine; D006639:Histidine; C028118:3-methylhistidine