Iwaki T, Iwaki A, Goldman J E
Department of Pathology, Columbia University College of Physicians and Surgeons, New York, NY 10032.
Acta Neuropathol. 1993;85(5):475-80. doi: 10.1007/BF00230485.
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.
αB-晶状体蛋白基因在脊椎动物晶状体中大量表达，在各种非晶状体组织中表达水平较低。在非晶状体组织中，αB-晶状体蛋白在心脏和骨骼肌中含量很高。使用针对αB-晶状体蛋白的特异性抗体，在人类骨骼肌活检中研究了αB-晶状体蛋白的细胞定位。在对NADH-四唑还原酶和细胞色素c氧化酶呈阳性反应的正常氧化肌纤维中观察到αB-晶状体蛋白的表达。在肌肉疾病中，在破碎红纤维中发现αB-晶状体蛋白的免疫反应性增加，这些纤维用琥珀酸脱氢酶组织化学染色呈深色。由于αB-晶状体蛋白与小热休克蛋白有关，并且可以由各种应激条件诱导，破碎红纤维中αB-晶状体蛋白免疫反应性的增加可能是由异常线粒体代谢产生的严重氧化应激所致。
