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Distinct Effects of Contraction-Induced Injury In Vivo on Four Different Murine Models of Dysferlinopathy
المؤلفون المشاركون
Roche, Joseph A.
Ru, Lisa W.
Bloch, Robert J.
المصدر
العدد
المجلد 2012، العدد 2012 (31 ديسمبر/كانون الأول 2012)، ص ص. 1-11، 11ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2012-02-06
دولة النشر
مصر
عدد الصفحات
11
التخصصات الرئيسية
الملخص 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.
نمط استشهاد جمعية علماء النفس الأمريكية (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
نمط استشهاد الجمعية الأمريكية للغات الحديثة (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
نمط استشهاد الجمعية الطبية الأمريكية (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
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-991462
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
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