Tubular aggregates in skeletal muscle: Just a special type of protein aggregates?
Tubular aggregates in skeletal muscle: Just a special type of protein aggregates?
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DOI：
10.1016/j.nmd.2011.10.005
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发表时间：
2012-03-01
影响因子：
2.8
通讯作者：
Schiaffino, Stefano
中科院分区：
文献类型：
--
作者：
Schiaffino, Stefano
管状聚集体是包涵体，通常在II型肌纤维和雄性中发现，由来自肌浆网的规则排列的小管组成。管状聚集体与多种肌肉病症相关，包括以无力和/或肌痛和/或痉挛为特征的定义不明确的“管状聚集体肌病”，并且也存在于不同的小鼠模型中，包括正常老化的肌肉。尚未确定诱导这些结构形成的机制，因为它们在小鼠体内的发育过程缓慢，需要数月。然而，相同的结构形成在几个小时内在大鼠肌肉保持在体外缺氧介质。在这里，我认为，管状聚集体的结果重塑肌浆网引起的错误折叠和聚集的膜蛋白，因此代表了一种特殊类型的“蛋白质聚集体”，由于改变蛋白质稳态。(C)2011 Elsevier B. V.保留所有权利。
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 "tubular aggregate myopathies" 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 "protein aggregates" due to altered proteostasis. (C) 2011 Elsevier B.V. All rights reserved.