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Duchenne型肌营养不良症骨骼肌凝集素受体异常分布及其意义

The abnormal distribution and significance of lectin receptors in skeletal muscle of the patients with duchenne mys-cular dystrophy (DMD).
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摘要 本文应用7种生物素化凝集素,借助ABC免疫酶染色技术,对16例Duchenne型肌营养不良症(DMD)患者和11例正常对照组的骨骼肌凝集素受体的定位和分布进行了研究。结果表明:DMD患者肌纤维膜上存在局灶性RCA-Ⅰ、WGA、ConA和PNA染色的阴性反应区,其发生率较正常对照组明显为高(P<0.01)。此外,可见一些小纤维的肌浆内RCA-Ⅰ、WGA和ConA染色阳性,以及增生结缔组织的WGA和PNA染色增强。同时,对发生这些改变的可能机制和意义进行简要的探讨。 In the present investigation, seven biotinlabelled lectins and avidin biotin-peroxidase complex were used to study the localisation and distribution of lectin receptors in skeletal muscle tissues of 16 patients with DMD and 11 normal controls. In DMD muscle, we found local absences in distribution of RCA-I. WGA. ConA and PNA receptors in theplasmalemma-basement membranes. The intracellular staining of small muscle fibers, was,observed with RCA-I. WGA and ConA. WGA and PNA staining apn parently increased in the proliferative endomysial and perimysial connective tissues. The results suggested that (1) the local absences of lectin receptors in muscle membrane might play an important role in pathogenesis of DMD; (2) the small muscle fibers with the intracellular positive staining might be regenerating fibers; (3) the increased lectin receptors in proliferative connective tissues might affect the myogenesis of the dystrophie human muscle.
出处 《中国神经精神疾病杂志》 CAS CSCD 北大核心 1991年第4期198-201,共4页 Chinese Journal of Nervous and Mental Diseases
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