Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle.
Alpha-dystrobrevin is a component of the dystrophin-glycoprotein complex (DGC) and is thought to have both structural and signaling roles in skeletal muscle. Mice deficient for alpha-dystrobrevin (adbn(-/-)) exhibit extensive myofiber degeneration and neuromuscular junction abnormalities. However, t...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Public Library of Science (PLoS)
2008-07-01
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| Series: | PLoS ONE |
| Online Access: | http://europepmc.org/articles/PMC2432020?pdf=render |
| _version_ | 1811273340613033984 |
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| author | Tina M Bunnell Michele A Jaeger Daniel P Fitzsimons Kurt W Prins James M Ervasti |
| author_facet | Tina M Bunnell Michele A Jaeger Daniel P Fitzsimons Kurt W Prins James M Ervasti |
| author_sort | Tina M Bunnell |
| collection | DOAJ |
| description | Alpha-dystrobrevin is a component of the dystrophin-glycoprotein complex (DGC) and is thought to have both structural and signaling roles in skeletal muscle. Mice deficient for alpha-dystrobrevin (adbn(-/-)) exhibit extensive myofiber degeneration and neuromuscular junction abnormalities. However, the biochemical stability of the DGC and the functional performance of adbn(-/-) muscle have not been characterized. Here we show that the biochemical association between dystrophin and beta-dystroglycan is compromised in adbn(-/-) skeletal muscle, suggesting that alpha-dystrobrevin plays a structural role in stabilizing the DGC. However, despite muscle cell death and DGC destabilization, costamere organization and physiological performance is normal in adbn(-/-) skeletal muscle. Our results demonstrate that myofiber degeneration alone does not cause functional deficits and suggests that more complex pathological factors contribute to the development of muscle weakness in muscular dystrophy. |
| first_indexed | 2024-04-12T22:57:22Z |
| format | Article |
| id | doaj.art-f44a2f19637d4a8fb17d315a17b8283d |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-04-12T22:57:22Z |
| publishDate | 2008-07-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-f44a2f19637d4a8fb17d315a17b8283d2022-12-22T03:13:10ZengPublic Library of Science (PLoS)PLoS ONE1932-62032008-07-0137e260410.1371/journal.pone.0002604Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle.Tina M BunnellMichele A JaegerDaniel P FitzsimonsKurt W PrinsJames M ErvastiAlpha-dystrobrevin is a component of the dystrophin-glycoprotein complex (DGC) and is thought to have both structural and signaling roles in skeletal muscle. Mice deficient for alpha-dystrobrevin (adbn(-/-)) exhibit extensive myofiber degeneration and neuromuscular junction abnormalities. However, the biochemical stability of the DGC and the functional performance of adbn(-/-) muscle have not been characterized. Here we show that the biochemical association between dystrophin and beta-dystroglycan is compromised in adbn(-/-) skeletal muscle, suggesting that alpha-dystrobrevin plays a structural role in stabilizing the DGC. However, despite muscle cell death and DGC destabilization, costamere organization and physiological performance is normal in adbn(-/-) skeletal muscle. Our results demonstrate that myofiber degeneration alone does not cause functional deficits and suggests that more complex pathological factors contribute to the development of muscle weakness in muscular dystrophy.http://europepmc.org/articles/PMC2432020?pdf=render |
| spellingShingle | Tina M Bunnell Michele A Jaeger Daniel P Fitzsimons Kurt W Prins James M Ervasti Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. PLoS ONE |
| title | Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. |
| title_full | Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. |
| title_fullStr | Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. |
| title_full_unstemmed | Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. |
| title_short | Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. |
| title_sort | destabilization of the dystrophin glycoprotein complex without functional deficits in alpha dystrobrevin null muscle |
| url | http://europepmc.org/articles/PMC2432020?pdf=render |
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