Tubular aggregates in skeletal muscle: Just a special type of protein aggregates?
- 作者：Stefano Schiaffino ; stefano.schiaffino@unipd.it
- 关键词：Tubular aggregates ; Skeletal muscle ; Myopathy ; Sarcoplasmic reticulum ; Protein aggregates ; Proteostasis
- 刊名：Neuromuscular Disorders
- 出版年：2012
- 期刊代码：203_09608966
- 类别：med
- 出版时间：March, 2012
- 卷：22
- 期：3
- 页码：199-207
- 文件大小：1153 K
摘要
Tubular aggregates are inclusions, usually found in type II muscle fibers and in males, consisting of regular arrays of tubules derived from the sarcoplasmic reticulum. Tubular aggregates are associated with a wide variety of muscle disorders, including poorly defined 鈥渢ubular aggregate myopathies鈥?characterized by weakness and/or myalgia and/or cramps, and are also present in different mouse models, including normal aging muscles. The mechanism(s) responsible for inducing the formation of these structures have not been identified, because of the slow time course of their development in vivo, several months in mice. However, identical structures are formed in a few hours in rat muscles kept in vitro in hypoxic medium. Here I suggest that tubular aggregates result from reshaping of sarcoplasmic reticulum caused by misfolding and aggregation of membrane proteins and thus represent a special type of 鈥減rotein aggregates鈥?due to altered proteostasis.