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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Valkova, Christina Rudolf, Rüdiger Betto, Romeo Röder, Ira V. Schwake, Michael Albrizio, Marina Kaether, Christoph |
| Description | Author Affiliation: Valkova C ( Leibniz Institut für Altersforschung-Fritz Lipmann Institut, 07743 Jena, Germany.); |
| Abstract | 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. Rer1 is expressed in the early secretory pathway in the myoblast line C2C12 and in mouse skeletal muscle, and up-regulated during myogenesis. Upon down-regulation of Rer1 in C2C12 cells, unassembled acetylcholine receptor -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 -subunits to the early secretory pathway. |
| ISSN | 00278424 |
| e-ISSN | 10916490 |
| Journal | Proceedings of the National Academy of Sciences of the United States of America |
| Issue Number | 2 |
| Volume Number | 108 |
| Language | English |
| Publisher | National Academy of Sciences |
| Publisher Date | 2011-01-01 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Cell Membrane Metabolism Endoplasmic Reticulum Membrane Glycoproteins Physiology Muscles Receptors, Cholinergic Receptors, Cytoplasmic And Nuclear Alleles Animals Down-Regulation Lysosomes Mice Mice, Transgenic Muscle, Skeletal Protein Transport Genetics Reverse Transcriptase Polymerase Chain Reaction Up-Regulation Research Support, Non-U.S. Gov't Multidisciplinary |
| Content Type | Text |
| Resource Type | Article |
| Subject | Multidisciplinary |
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