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肌肉与红细胞膜之间的生化关系。关于杜氏肌营养不良症的解读

Biochemical relationships between muscle and erythrocyte membrane. Interpretations with respect to Duchenne muscular dystrophy.

作者信息

Roses A D, Herbstreith M H, Metcalf B S, Hartwig G B

出版信息

Electroencephalogr Clin Neurophysiol Suppl. 1978(34):463-8.

PMID:285848
Abstract

Whether Duchenne muscular dystrophy (DMD) is a disease of primary myogenic, secondary neuropathic, vascular or membrane etiology is the subject of some debate. Using the erythrocyte membrane as a biopsy tissue we present biochemical data that support the possibility of a defect in myosin as the genetic defect in DMD. Peptide analysis of hydrolyzed erythrocyte spectrin supports previous data demonstrating an alteration in DMD spectrin. The biochemical and biophysical similarities between spectrin and myosin can be tested with available technology. The hypothesis that a defect in myosin may be responsible for DMD is attractive because it is testable.

摘要

杜氏肌营养不良症(DMD)是原发性肌源性、继发性神经源性、血管性还是膜性病因的疾病,存在一些争议。我们以红细胞膜作为活检组织,提供了生化数据,支持肌球蛋白缺陷可能是DMD遗传缺陷的可能性。水解红细胞血影蛋白的肽分析支持了先前证明DMD血影蛋白改变的数据。血影蛋白和肌球蛋白之间的生化和生物物理相似性可以用现有技术进行测试。肌球蛋白缺陷可能导致DMD的假说很有吸引力,因为它是可检验的。

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