کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
6021679 | 1580650 | 2015 | 7 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Calreticulin levels determine onset of early muscle denervation by fast motoneurons of ALS model mice
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
عصب شناسی
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![عکس صفحه اول مقاله: Calreticulin levels determine onset of early muscle denervation by fast motoneurons of ALS model mice Calreticulin levels determine onset of early muscle denervation by fast motoneurons of ALS model mice](/preview/png/6021679.png)
چکیده انگلیسی
Although the precise signaling mechanisms underlying the vulnerability of some sub-populations of motoneurons in ALS remain unclear, critical factors such as metallo-proteinase 9 expression, neuronal activity and endoplasmic reticulum stress have been shown to be involved. In the context of SOD1G93A ALS mouse model, we previously showed that a two-fold decrease in calreticulin (CRT) is occurring in the vulnerable fast motoneurons. Here, we asked to which extent the decrease in CRT levels was causative to muscle denervation and/or motoneuron degeneration. Toward this goal, a hemizygous deletion of the crt gene in SOD1G93A mice was generated since the complete ablation of crt is embryonic lethal. We observed that SOD1G93A;crt+/â mice display increased and earlier muscle weakness and muscle denervation compared to SOD1G93A mice. While CRT reduction in motoneurons leads to a strong upregulation of two factors important in motoneuron dysfunction, ER stress and mTOR activation, it does not aggravate motoneuron death. Our results underline a prevalent role for CRT levels in the early phase of muscle denervation and support CRT regulation as a potential therapeutic approach.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neurobiology of Disease - Volume 73, January 2015, Pages 130-136
Journal: Neurobiology of Disease - Volume 73, January 2015, Pages 130-136
نویسندگان
Nathalie Bernard-Marissal, Claire Sunyach, Thomas Marissal, Cédric Raoul, Brigitte Pettmann,