ALTERATIONS OF PROTEIN-DEGRADATION AND 2-D PROTEIN PATTERN IN MUSCLE-CELLS OF MDX AND DMD ORIGIN
ALTERATIONS OF PROTEIN-DEGRADATION AND 2-D PROTEIN PATTERN IN MUSCLE-CELLS OF MDX AND DMD ORIGIN
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DOI：
10.1016/0006-291x(92)90242-d
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发表时间：
1992-12-30
影响因子：
3.1
通讯作者：
RODEMANN, HP
中科院分区：
文献类型：
--
作者：
KAMPER, A;RODEMANN, HP
通过[35 S]蛋氨酸脉冲追踪实验和随后的高分辨率二维凝胶电泳测定MDX、DMD和正常肌肉的细胞内蛋白质周转。在MDX肌管中，短寿命和长寿命蛋白的细胞内降解显著增加了1,4- 2,1倍。在野生型中，短寿命蛋白的降解率约为2.6% /h，而在MDX中，这些蛋白的降解率为5.7% /h。在野生型中，长寿命蛋白的降解速率为1.8% /h，在MDX中，降解速率为2.5% /h。此外，我们还描述了一种51000 Da的蛋白质，IEP为5.1 (p 51 5.1)，其净含量在培养的MDX和DMD肌肉细胞以及分离的MDX肌纤维中高度特异性地降低。与对照组相比，钙通道阻滞剂丹曲林和维拉帕米治疗可抑制MDX肌管中p515.1的降解90%以上。
Intracellular protein turnover of MDX, DMD and normal muscle was determined by [35 S] methionine pulse-chase experiments and subsequent high resolution 2-D gel electrophoresis. In MDX myotubes intracellular degradation of short-lived and long-lived proteins was markedly increased by a factor of 1, 4-2, 1. In wildtype the rate of degradation of short-lived proteins was approximatly 2, 6%/h, whereas in MDX these proteins were degraded by 5, 7%/h. Long-lived proteins were degraded in wildtype at a rate of 1, 8%/h, and in MDX at a rate of 2, 5%/h. Furthermore, we have described a 51.000 Da protein with an IEP of 5.1 (p 51 5.1), whose net content is highly and specifically reduced in cultured MDX and DMD muscle cells as well as in isolated MDX muscle fibers. Treatment with calcium-channel blockers Dantrolene and Verapamil inhibited the degradation of p 51 5.1 in MDX myotubes by more than 90% in contrast to controls.