Fast muscle fibers are preferentially affected in Duchenne muscular dystrophy.
We show that Duchenne muscular dystrophy (DMD) selectively affects a subset of skeletal muscle fibers specialized for fast contraction. Muscle fiber types were characterized immunohistochemically with monoclonal antibodies that distinguish isoforms of fetal and adult-fast or adult-slow myosin heavy chain present in the same fiber. Fetal myosin expression increased with patient age and was not due to arrested development but rather to de novo synthesis, which served as a sensitive indicator of muscle regeneration. A subset of fast fibers were the first to degenerate (type IIb). Extensive fast fiber regeneration occurred before slow fibers were affected. These results suggest that the DMD gene product has a specific function in a subpopulation of muscle fibers specialized to respond to the highest frequency of neuronal stimulation with maximal rates of contraction.
Webster
C
C
Department of Pharmacology, Stanford University School of Medicine, California 94305.
Silberstein
L
L
Hays
A P
AP
Blau
H M
HM
D000367:Age Factors; D000911:Antibodies, Monoclonal; D006801:Humans; D009119:Muscle Contraction; D009132:Muscles / Q000473:pathology*; D009136:Muscular Dystrophies / Q000473:pathology*; D009218:Myosins / Q000378:metabolism
D000911:Antibodies, Monoclonal; D009218:Myosins