التفاصيل البيبلوغرافية
| العنوان: |
Sorting receptor Rer1 controls surface expression of muscle acetylcholine receptors by ER retention of unassembled α-subunits. |
| المؤلفون: |
Valkova, Christina, Albrizio, Marina, Röder, Ira V., Schwake, Michael, Betto, Romeo, Rudolf, Rüdiger, Kaether, Christoph |
| المصدر: |
Proceedings of the National Academy of Sciences of the United States of America; 1/11/2011, Vol. 108 Issue 2, p621-625, 5p, 5 Diagrams |
| مصطلحات موضوعية: |
HUMAN molecular genetics, CELL membranes, ACETYLCHOLINE, MUSCARINIC agonists, MYOGENESIS |
| مستخلص: |
The nicotinic acetylcholine receptor of skeletal muscle is composed of five subunits that are assembled in a stepwise manner. Quality control mechanisms ensure that only fully assembled receptors reach the cell surface. Here, we show that Rer1, a putative Golgi-ER retrieval receptor, is involved in the biogenesis of acetylcholine receptors. Rerl is expressed in the early secretory pathway in the myoblast line C2C12 and in mouse skeletal muscle, and upregulated during myogenesis. Upon down-regulation of Rerl in C2C12 cells, unassembled acetylcholine receptor α-subunits escape from the ER and are transported to the plasma membrane and lysosomes, where they are degraded. As a result, the amount of fully assembled receptor at the cell surface is reduced. In vivo Rer1 knockdown and genetic inactivation of one Rer1 allele lead to significantly smaller neuromuscular junctions in mice. Our data show that Rer1 is a functionally important unique factor that controls surface expression of muscle acetylcholine receptors by localizing unassembled α-subunits to the early secretory pathway. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
Complementary Index |
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