Cargando…

Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse

Syncoilin is a 64-kDa intermediate filament protein expressed in skeletal muscle and enriched at the perinucleus, sarcolemma, and myotendinous and neuromuscular junctions. Due to its pattern of cellular localization and binding partners, syncoilin is an ideal candidate to be both an important struct...

Descripción completa

Detalles Bibliográficos
Autores principales: McCullagh, Karl J. A., Edwards, Ben, Kemp, Matthew W., Giles, Laura C., Burgess, Matthew, Davies, Kay E.
Formato: Texto
Lenguaje:English
Publicado: Springer-Verlag 2008
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2515546/
https://www.ncbi.nlm.nih.gov/pubmed/18594912
http://dx.doi.org/10.1007/s00335-008-9120-2
_version_ 1782158430228709376
author McCullagh, Karl J. A.
Edwards, Ben
Kemp, Matthew W.
Giles, Laura C.
Burgess, Matthew
Davies, Kay E.
author_facet McCullagh, Karl J. A.
Edwards, Ben
Kemp, Matthew W.
Giles, Laura C.
Burgess, Matthew
Davies, Kay E.
author_sort McCullagh, Karl J. A.
collection PubMed
description Syncoilin is a 64-kDa intermediate filament protein expressed in skeletal muscle and enriched at the perinucleus, sarcolemma, and myotendinous and neuromuscular junctions. Due to its pattern of cellular localization and binding partners, syncoilin is an ideal candidate to be both an important structural component of myocytes and a potential mediator of inherited myopathies. Here we present a report of a knockout mouse model for syncoilin and the results of an investigation into the effect of a syncoilin null state on striated muscle function in 6–8-week-old mice. An analysis of proteins known to associate with syncoilin showed that ablation of syncoilin had no effect on absolute expression or spatial localization of desmin or alpha dystrobrevin. Our syncoilin-null animal exhibited no differences in cardiotoxin-induced muscle regeneration, voluntary wheel running, or enforced treadmill exercise capacity, relative to wild-type controls. Finally, a mechanical investigation of isolated soleus and extensor digitorum longus indicated a potential differential reduction in muscle strength and resilience. We are the first to present data identifying an increased susceptibility to muscle damage in response to an extended forced exercise regime in syncoilin-deficient muscle. This study establishes a second viable syncoilin knockout model and highlights the importance of further investigations to determine the role of syncoilin in skeletal muscle.
format Text
id pubmed-2515546
institution National Center for Biotechnology Information
language English
publishDate 2008
publisher Springer-Verlag
record_format MEDLINE/PubMed
spelling pubmed-25155462008-08-14 Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse McCullagh, Karl J. A. Edwards, Ben Kemp, Matthew W. Giles, Laura C. Burgess, Matthew Davies, Kay E. Mamm Genome Article Syncoilin is a 64-kDa intermediate filament protein expressed in skeletal muscle and enriched at the perinucleus, sarcolemma, and myotendinous and neuromuscular junctions. Due to its pattern of cellular localization and binding partners, syncoilin is an ideal candidate to be both an important structural component of myocytes and a potential mediator of inherited myopathies. Here we present a report of a knockout mouse model for syncoilin and the results of an investigation into the effect of a syncoilin null state on striated muscle function in 6–8-week-old mice. An analysis of proteins known to associate with syncoilin showed that ablation of syncoilin had no effect on absolute expression or spatial localization of desmin or alpha dystrobrevin. Our syncoilin-null animal exhibited no differences in cardiotoxin-induced muscle regeneration, voluntary wheel running, or enforced treadmill exercise capacity, relative to wild-type controls. Finally, a mechanical investigation of isolated soleus and extensor digitorum longus indicated a potential differential reduction in muscle strength and resilience. We are the first to present data identifying an increased susceptibility to muscle damage in response to an extended forced exercise regime in syncoilin-deficient muscle. This study establishes a second viable syncoilin knockout model and highlights the importance of further investigations to determine the role of syncoilin in skeletal muscle. Springer-Verlag 2008-07-02 2008 /pmc/articles/PMC2515546/ /pubmed/18594912 http://dx.doi.org/10.1007/s00335-008-9120-2 Text en © The Author(s) 2008 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.
spellingShingle Article
McCullagh, Karl J. A.
Edwards, Ben
Kemp, Matthew W.
Giles, Laura C.
Burgess, Matthew
Davies, Kay E.
Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse
title Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse
title_full Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse
title_fullStr Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse
title_full_unstemmed Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse
title_short Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse
title_sort analysis of skeletal muscle function in the c57bl6/sv129 syncoilin knockout mouse
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2515546/
https://www.ncbi.nlm.nih.gov/pubmed/18594912
http://dx.doi.org/10.1007/s00335-008-9120-2
work_keys_str_mv AT mccullaghkarlja analysisofskeletalmusclefunctioninthec57bl6sv129syncoilinknockoutmouse
AT edwardsben analysisofskeletalmusclefunctioninthec57bl6sv129syncoilinknockoutmouse
AT kempmattheww analysisofskeletalmusclefunctioninthec57bl6sv129syncoilinknockoutmouse
AT gileslaurac analysisofskeletalmusclefunctioninthec57bl6sv129syncoilinknockoutmouse
AT burgessmatthew analysisofskeletalmusclefunctioninthec57bl6sv129syncoilinknockoutmouse
AT davieskaye analysisofskeletalmusclefunctioninthec57bl6sv129syncoilinknockoutmouse