Cell and fiber-type distribution of dystrophin.
Duchenne muscular dystrophy is the result of dystrophin deficiency. We have determined the cell types likely to express the pathogenic effects of this neuromuscular disease by determining the pattern of dystrophin expression in normal cells. We find that all physiological types of muscle cells express dystrophin at similar levels, and that the dystrophin content of various tissues correlates with the myogenic cell population of each tissue. The dystrophin content of brain and spinal cord, however, is found not to correlate with any type of muscle cell, and it is suggested that neurons express dystrophin. The potential involvement of striated muscle fibers, the vasculature, and the nervous system in the etiology of Duchenne muscular dystrophy makes it likely that the disease is a complex disorder of combined pathogenesis. We also find that the dystrophic chicken does not represent an animal model for dystrophin deficiency.
Hoffman
E P
EP
Division of Genetics, Children's Hospital, Boston, Massachusetts.
Hudecki
M S
MS
Rosenberg
P A
PA
Pollina
C M
CM
Kunkel
L M
LM
D000818:Animals; D000918:Antibody Specificity; D000936:Antigen-Antibody Complex / Q000032:analysis; D015153:Blotting, Western; D001923:Brain Chemistry; D002478:Cells, Cultured; D002540:Cerebral Cortex / Q000032:analysis*; D002645:Chickens; D002846:Chromatography, Affinity; D016189:Dystrophin; D051379:Mice; D009124:Muscle Proteins / Q000032:analysis* / Q000502:physiology; D009130:Muscle, Smooth / Q000032:analysis; D009132:Muscles / Q000032:analysis*; D009137:Muscular Dystrophy, Animal / Q000378:metabolism; D009457:Neuroglia / Q000032:analysis; D009474:Neurons / Q000032:analysis; D009928:Organ Specificity; D051381:Rats; D013116:Spinal Cord / Q000032:analysis*
D000936:Antigen-Antibody Complex; D016189:Dystrophin; D009124:Muscle Proteins