杜氏肌营养不良症患者培养的成肌细胞和肌管中的胶原蛋白合成

Collagen synthesis in cultured myoblasts and myotubes from patients with Duchenne muscular dystrophy.

作者信息

de Jong L, Wolterman R A, Hillarius S, Bolhuis P A

机构信息

Laboratory of Biochemistry, University of Amsterdam, The Netherlands.

出版信息

J Neurol Sci. 1987 Dec;82(1-3):271-80. doi: 10.1016/0022-510x(87)90023-2.

DOI:10.1016/0022-510x(87)90023-2

PMID:3440869

Abstract

Collagen synthesis was studied in cultured myoblasts and myotubes from 4 patients with Duchenne muscular dystrophy (DMD) and 4 control persons. Incorporation of [3H]proline into collagen of DMD cells and control cells was not significantly different. The same types of collagen, i.e., type I and type III were synthesized by myogenic cells from DMD patients and controls. In contrast to others, we could not obtain evidence for an increase in the degree of prolyl hydroxylation in collagen of the DMD muscle cells.

摘要

对4例杜氏肌营养不良症（DMD）患者和4例对照者的培养成肌细胞和肌管中的胶原蛋白合成进行了研究。[3H]脯氨酸掺入DMD细胞和对照细胞胶原蛋白中的情况无显著差异。DMD患者和对照者的成肌细胞合成的胶原蛋白类型相同，即I型和III型。与其他研究结果不同的是，我们未能获得证据证明DMD肌肉细胞胶原蛋白中脯氨酰羟化程度增加。