Alpha B-crystallin in oxidative muscle fibers and its accumulation in ragged-red fibers: a comparative immunohistochemical and histochemical study in human skeletal muscle.
The alpha B-crystallin gene is abundantly expressed in the vertebrate lens and at lower levels in various non-lenticular tissues. Among the non-lenticular tissues, alpha B-crystallin is present at high levels in the heart and skeletal muscle. Using a specific antibody against alpha B-crystallin, the cellular localization of alpha B-crystallin was studied in biopsies of human skeletal muscles. Expression of alpha B-crystallin was observed in normal oxidative muscle fibers that show positive reactions for NADH-tetrazolium reductase and cytochrome c oxidase. In muscle diseases increased immunoreactivity for alpha B-crystallin was found in ragged-red fibers, which stained darkly with histochemistry for succinate dehydrogenase. Since alpha B-crystallin is related to small heat-shock proteins and can be induced by various stress conditions, the increased alpha B-crystallin immunoreactivity of ragged-red fibers could result from profound oxidative stress produced by the abnormal mitochondrial metabolism.
Department of Pathology, Columbia University College of Physicians and Surgeons, New York, NY 10032.
T
T
Iwaki
A
A
Iwaki
J E
JE
Goldman
D000328:Adult; D003459:Crystallins / Q000378:metabolism*; D003576:Electron Transport Complex IV / Q000276:immunology / Q000378:metabolism; D005260:Female; D006360:Heat-Shock Proteins / Q000276:immunology / Q000378:metabolism; D006651:Histocytochemistry; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000276:immunology / Q000378:metabolism; D017240:Mitochondrial Myopathies / Q000473:pathology; D009132:Muscles / Q000294:innervation / Q000378:metabolism* / Q000648:ultrastructure; D009206:Myocardium / Q000378:metabolism / Q000648:ultrastructure; D009246:NADH Tetrazolium Reductase / Q000378:metabolism; D010084:Oxidation-Reduction; D013385:Succinate Dehydrogenase / Q000276:immunology / Q000378:metabolism
D003459:Crystallins; D006360:Heat-Shock Proteins; D013385:Succinate Dehydrogenase; D009246:NADH Tetrazolium Reductase; D003576:Electron Transport Complex IV