Distinct Effects of Contraction-Induced Injury In Vivo on Four Different Murine Models of Dysferlinopathy
Joint Authors
Roche, Joseph A.
Ru, Lisa W.
Bloch, Robert J.
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-11, 11 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-02-06
Country of Publication
Egypt
No. of Pages
11
Main Subjects
Abstract EN
Mutations in the DYSF gene, encoding dysferlin, cause muscular dystrophies in man.
We compared 4 dysferlinopathic mouse strains: SJL/J and B10.SJL-Dysfim/AwaJ (B10.SJL), and A/J and B6.A-Dysfprmd/GeneJ (B6.A/J).
The former but not the latter two are overtly myopathic and weaker at 3 months of age.
Following repetitive large-strain injury (LSI) caused by lengthening contractions, all except B6.A/J showed ~40% loss in contractile torque.
Three days later, torque in SJL/J, B10.SJL and controls, but not A/J, recovered nearly completely.
B6.A/J showed ~30% torque loss post-LSI and more variable recovery.
Pre-injury, all dysferlinopathic strains had more centrally nucleated fibers (CNFs) and all but A/J showed more inflammation than controls.
At D3, all dysferlinopathic strains showed increased necrosis and inflammation, but not more CNFs; controls were unchanged.
Dystrophin-null DMDmdx mice showed more necrosis and inflammation than all dysferlin-nulls.
Torque loss and inflammation on D3 across all strains were linearly related to necrosis.
Our results suggest that (1) dysferlin is not required for functional recovery 3 days after LSI; (2) B6.A/J mice recover from LSI erratically; (3) SJL/J and B10.SJL muscles recover rapidly, perhaps due to ongoing myopathy; (4) although they recover function to different levels, all 4 dysferlinopathic strains show increased inflammation and necrosis 3 days after LSI.
American Psychological Association (APA)
Roche, Joseph A.& Ru, Lisa W.& Bloch, Robert J.. 2012. Distinct Effects of Contraction-Induced Injury In Vivo on Four Different Murine Models of Dysferlinopathy. BioMed Research International،Vol. 2012, no. 2012, pp.1-11.
https://search.emarefa.net/detail/BIM-991462
Modern Language Association (MLA)
Roche, Joseph A.…[et al.]. Distinct Effects of Contraction-Induced Injury In Vivo on Four Different Murine Models of Dysferlinopathy. BioMed Research International No. 2012 (2012), pp.1-11.
https://search.emarefa.net/detail/BIM-991462
American Medical Association (AMA)
Roche, Joseph A.& Ru, Lisa W.& Bloch, Robert J.. Distinct Effects of Contraction-Induced Injury In Vivo on Four Different Murine Models of Dysferlinopathy. BioMed Research International. 2012. Vol. 2012, no. 2012, pp.1-11.
https://search.emarefa.net/detail/BIM-991462
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-991462