Morphological and functional analyses of skeletal muscles from an immunodeficient animal model of limb-girdle muscular dystrophy type 2E

Muscle & Nerve
Gaia GiovannelliMaurilio Sampaolesi

Abstract

Limb-girdle muscular dystrophy type 2E (LGMD2E) is caused by mutations in the β-sarcoglycan gene, which is expressed in skeletal, cardiac, and smooth muscles. β-Sarcoglycan-deficient (Sgcb-null) mice develop severe muscular dystrophy and cardiomyopathy with focal areas of necrosis. In this study we performed morphological (histological and cellular characterization) and functional (isometric tetanic force and fatigue) analyses in dystrophic mice. Comparison studies were carried out in 1-month-old (clinical onset of the disease) and 7-month-old control mice (C57Bl/6J, Rag2/γc-null) and immunocompetent and immunodeficient dystrophic mice (Sgcb-null and Sgcb/Rag2/γc-null, respectively). We found that the lack of an immunological system resulted in an increase of calcification in striated muscles without impairing extensor digitorum longus muscle performance. Sgcb/Rag2/γc-null muscles showed a significant reduction of alkaline phosphate-positive mesoangioblasts. The immunological system counteracts skeletal muscle degeneration in the murine model of LGMD2E. Muscle Nerve, 2018.

References

Oct 1, 1988·The Journal of Physiology·S V Brooks, J A Faulkner
Jun 1, 1988·Journal of Applied Physiology·C Y Seow, N L Stephens
Aug 1, 1993·The Journal of Cell Biology·J M Ervasti, K P Campbell
Nov 1, 1996·Human Molecular Genetics·E M McNallyE P Hoffman
Jul 6, 2000·Laboratory Investigation; a Journal of Technical Methods and Pathology·J MorrisonG Bou-Gharios
Feb 13, 2001·Clinical Immunology : the Official Journal of the Clinical Immunology Society·M J SpencerJ G Tidball
Dec 19, 2001·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Guofeng RenNikolaos G Frangogiannis
Jun 22, 2002·Current Opinion in Genetics & Development·Madeleine Durbeej, Kevin P Campbell
Aug 19, 2003·Trends in Cardiovascular Medicine·Matthew T Wheeler, Elizabeth M McNally
Jan 14, 1964·Proceedings of the Royal Society of London. Series B, Containing Papers of a Biological Character·A V HILL
Jul 20, 2004·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Marcin DobaczewskiNikolaos G Frangogiannis
Aug 3, 2004·Nature Reviews. Immunology·Thomas A Wynn
Aug 28, 2004·Journal of Cell Science·Enrico TagliaficoStefano Ferrari
Sep 6, 2005·Science·Didier MontarrasMargaret Buckingham
Nov 17, 2006·Nature·Maurilio SampaolesiGiulio Cossu
Dec 13, 2006·Journal of the American College of Cardiology·Pawel ZymekNikolaos G Frangogiannis
Feb 13, 2007·Nature Cell Biology·Arianna DellavalleGiulio Cossu
Jan 19, 2010·Nature Cell Biology·Aaron W B JoeFabio M V Rossi
Mar 3, 2010·Journal of Muscle Research and Cell Motility·Marco CassanoMaurilio Sampaolesi
Jul 16, 2010·Neuromuscular Disorders : NMD·M C E HermansC G Faber
Jun 29, 2011·The Journal of Cell Biology·Stefania CrippaMaurilio Sampaolesi
May 11, 2012·The Journal of Immunology : Official Journal of the American Association of Immunologists·Lidia BosurgiPatrizia Rovere-Querini
Mar 20, 2013·Mediators of Inflammation·Yacine KharrazPura Muñoz-Cánoves
Aug 27, 2013·Molecular Therapy : the Journal of the American Society of Gene Therapy·Denis ValleseVincent Mouly
Nov 21, 2013·The Journal of Pathology·Margaret L NovakTimothy J Koh
Aug 8, 2015·Nature Medicine·Helen M BlauAndrew T V Ho
Nov 17, 2015·The Journal of Clinical Investigation·Mattia QuattrocelliMaurilio Sampaolesi
Apr 19, 2016·Stem Cell Research & Therapy·Aleksandra MaziarzAgnieszka Banas
May 4, 2016·The Journal of Clinical Investigation·Manabu ShiraishiKen Suzuki

❮ Previous
Next ❯

Citations

Aug 20, 2020·Cell Death & Disease·Nefele GiarratanaMaurilio Sampaolesi
Mar 8, 2019·International Journal of Molecular Sciences·Jordi CampsMaurilio Sampaolesi
Apr 4, 2021·International Journal of Molecular Sciences·Carles Sánchez RieraLuca Madaro

❮ Previous
Next ❯

Methods Mentioned

BETA
biopsies
FACS

Software Mentioned

ImageJ
GraphPad
GraphPad Prism

Related Concepts

Related Feeds

Cardiomyopathy

Cardiomyopathy is a disease of the heart muscle, that can lead to muscular or electrical dysfunction of the heart. It is often an irreversible disease that is associated with a poor prognosis. There are different causes and classifications of cardiomyopathies. Here are the latest discoveries pertaining to this disease.