Genetic compensation for sarcoglycan loss by integrin alpha7beta1 in muscle

Journal of Cell Science
Michael J AllikianE M McNally

Abstract

Disruption of the sarcoglycan complex leads to muscle membrane instability and muscular dystrophy in humans and mice. Through the dystrophin glycoprotein complex, sarcoglycan participates in connecting the internal cytoskeleton to the membrane and the extracellular matrix. Integrin alpha7beta1 is also a transmembrane protein of skeletal and cardiac muscle that similarly links the cytoskeleton to the extracellular matrix. Mice lacking integrin alpha7 develop mild muscle degeneration, while sarcoglycan mutant mice display overt muscle degeneration and muscular dystrophy. In sarcoglycan-deficient muscle, integrin alpha7 protein was upregulated at the plasma membrane. To ascertain whether integrin alpha7 upregulation compensates for the loss of the transmembrane sarcoglycan linkage in sarcoglycan-deficient muscle, we generated mice lacking both integrin alpha7 and gamma-sarcoglycan (gxi). These double-mutant gxi mice exhibit profound, rapid muscle degeneration leading to death before one month of age consistent with a weakened cellular attachment to the extracellular matrix. The regenerative capacity of gxi muscle was intact with increased embryonic myosin heavy chain expression, myofiber central nucleation and normal in vivo myobl...Continue Reading

References

Dec 1, 1991·The Journal of Cell Biology·K Ohlendieck, K P Campbell
Aug 1, 1995·Genes & Development·R Fässler, M Meyer
Jun 7, 1994·Proceedings of the National Academy of Sciences of the United States of America·H XuE Engvall
Aug 1, 1993·The Journal of Cell Biology·J M Ervasti, K P Campbell
Apr 15, 1993·Proceedings of the National Academy of Sciences of the United States of America·B J PetrofH L Sweeney
Aug 25, 1997·Experimental Cell Research·S CrawleyS J Kaufman
Oct 23, 1997·The Journal of Cell Biology·V StraubK P Campbell
Nov 14, 1997·Nature Genetics·U MayerK von der Mark
Jan 1, 1997·Methods in Cell Biology·T A Rando, H M Blau
May 20, 1998·Nature Genetics·Y K HayashiK Arahata
Aug 26, 1998·The Journal of Clinical Investigation·W KuangE Engvall
Sep 23, 1998·The Journal of Cell Biology·F DuclosK P Campbell
Nov 12, 1998·The American Journal of Pathology·V StraubK P Campbell
Nov 13, 1998·The Journal of Cell Biology·D TavernaR O Hynes
Oct 6, 1999·Current Opinion in Cell Biology·M D Henry, K P Campbell
Jan 12, 2000·The Journal of Cell Biology·T G ThompsonL M Kunkel
Feb 19, 2000·Microscopy Research and Technique·J A Rafael, S C Brown
Feb 19, 2000·Microscopy Research and Technique·A A HackE M McNally
Sep 27, 2000·Human Molecular Genetics·V Allamand, K P Campbell
Apr 17, 2001·Brain Pathology·E OzawaI Nonaka
May 18, 2001·Current Opinion in Cardiology·A HeydemannE M McNally
May 23, 2001·Neuromuscular Disorders : NMD·M KääriäinenH Kalimo
Nov 24, 2001·Trends in Cell Biology·M A Schwartz
Jan 16, 2002·Current Opinion in Genetics & Development·Alan K HoweR L Juliano
Mar 5, 2002·Nature Medicine·Scott Q HarperJeffrey S Chamberlain
Dec 12, 2002·The Journal of Endocrinology·H H A G M van der PuttenM E Everts
May 10, 2003·Developmental Cell·Martin SchwanderUlrich Müller

❮ Previous
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Citations

Sep 11, 2012·Analytical and Bioanalytical Chemistry·Neha AroraEmily A Smith
Sep 14, 2006·Journal of Human Genetics·Motoi Kanagawa, Tatsushi Toda
Dec 1, 2011·Purinergic Signalling·Rosa MancinelliStefania Fulle
Sep 29, 2009·Expert Reviews in Molecular Medicine·Dorianna Sandonà, Romeo Betto
Sep 15, 2006·Nature Reviews. Molecular Cell Biology·Kay E Davies, Kristen J Nowak
Oct 13, 2006·The Journal of Experimental Medicine·Ganapati H MahabeleshwarTatiana V Byzova
Dec 20, 2011·Human Molecular Genetics·Jianming LiuStephen J Kaufman
Aug 4, 2012·Human Molecular Genetics·Tamar E SztalPeter D Currie
Dec 13, 2006·Cell Structure and Function·Reina Hashimoto, Masamitsu Yamaguchi
Sep 24, 2008·Annual Review of Physiology·Gregory Q Wallace, Elizabeth M McNally
Nov 15, 2013·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Marta PellegattaMaria Laura Feltri
Mar 15, 2014·Molecular Medicine Reports·Omar A Mahmood, Xin Mei Jiang
Feb 5, 2013·Mitochondrion·T O KragJ Vissing
Dec 17, 2008·The American Journal of Pathology·Jachinta E RooneyDean J Burkin
Dec 17, 2008·Journal of Controlled Release : Official Journal of the Controlled Release Society·Younghoon KimDennis E Discher
Mar 7, 2007·The International Journal of Biochemistry & Cell Biology·Nathalie DanièleMarc Bartoli
May 4, 2007·Neuromuscular Disorders : NMD·Laurin M HanftJames M Ervasti
Jan 27, 2007·The American Journal of Pathology·Derek J Milner, Stephen J Kaufman
May 28, 2005·Journal of Cellular Physiology·Alessandro Sgambato, Andrea Brancaccio
Jun 7, 2005·Muscle & Nerve·Eijiro OzawaMikiharu Yoshida
Jun 29, 2007·Glia·Michael A ChernousovDavid J Carey
Nov 19, 2005·Scandinavian Journal of Medicine & Science in Sports·M D GroundsJ White
Oct 2, 2014·International Journal of Experimental Pathology·Charlotte Whitmore, Jennifer Morgan
Jan 22, 2015·Human Molecular Genetics·Janelle M SpinazzolaElisabeth R Barton
Mar 27, 2007·Biochimica Et Biophysica Acta·Jiwei ChenYiu-mo Michael Chan
Aug 19, 2006·Biochimica Et Biophysica Acta·Ahlke HeydemannElizabeth M McNally
Jan 8, 2014·The American Journal of Pathology·Kinga I GawlikMadeleine Durbeej
Apr 27, 2005·The American Journal of Sports Medicine·Tero A H JärvinenMarkku Järvinen
Nov 12, 2005·American Journal of Physiology. Cell Physiology·Jun-Tzu ChaoEmily Wilson
May 26, 2007·Journal of Applied Physiology·W W DyeJ D Humphrey
Jul 5, 2006·Genes & Development·Xiaozhong Shi, Daniel J Garry
Sep 27, 2016·ELife·Davy VanhoutteJeffery D Molkentin
Jan 20, 2006·American Journal of Physiology. Cell Physiology·Marni D BoppartStephen J Kaufman
Nov 13, 2007·American Journal of Physiology. Lung Cellular and Molecular Physiology·Pawan SharmaAndrew J Halayko

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