spacer gif spacer gif spacer gif spacer gif spacer gif
QUICK SEARCH:   [advanced]


spacer gif
     Home     Help     Feedback     Subscriptions     Archive     Search     Table of Contents    

First published online 9 May 2006
doi: 10.1242/jcs.02952

Journal of Cell Science 119, 2185-2195 (2006)
Published by The Company of Biologists 2006
This Article
Right arrow Figures Only
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Supplementary Material
Right arrow All Versions of this Article:
jcs.02952v1
119/11/2185    most recent
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Rooney, J. E.
Right arrow Articles by Burkin, D. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Rooney, J. E.
Right arrow Articles by Burkin, D. J.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

Research Article

Severe muscular dystrophy in mice that lack dystrophin and {alpha}7 integrin

Jachinta E. Rooney1,*, Jennifer V. Welser1,*, Melissa A. Dechert1, Nichole L. Flintoff-Dye1, Stephen J. Kaufman2 and Dean J. Burkin1,3,{ddagger}

1 Department of Pharmacology, University of Nevada, Reno, NV 89557, USA
2 Department of Cell and Developmental Biology, University of Illinois, Urbana, IL 61801, USA
3 Nevada Transgenic Center, University of Nevada, Reno, NV 89557, USA

{ddagger} Author for correspondence (e-mail: dburkin{at}med.unr.edu)

Accepted 21 February 2006

The dystrophin glycoprotein complex links laminin in the extracellular matrix to the cell cytoskeleton. Loss of dystrophin causes Duchenne muscular dystrophy, the most common human X-chromosome-linked genetic disease. The {alpha}7ß1 integrin is a second transmembrane laminin receptor expressed in skeletal muscle. Mutations in the {alpha}7 integrin gene cause congenital myopathy in humans and mice. The {alpha}7ß1 integrin is increased in the skeletal muscle of Duchenne muscular dystrophy patients and mdx mice. This observation has led to the suggestion that dystrophin and {alpha}7ß1 integrin have complementary functional and structural roles. To test this hypothesis, we generated mice lacking both dystrophin and {alpha}7 integrin (mdx/{alpha}7-/-). The mdx/{alpha}7-/- mice developed early-onset muscular dystrophy and died at 2-4 weeks of age. Muscle fibers from mdx/{alpha}7-/- mice exhibited extensive loss of membrane integrity, increased centrally located nuclei and inflammatory cell infiltrate, greater necrosis and increased muscle degeneration compared to mdx or {alpha}7-integrin null animals. In addition, loss of dystrophin and/or {alpha}7 integrin resulted in altered expression of laminin-{alpha}2 chain. These results point to complementary roles for dystrophin and {alpha}7ß1 integrin in maintaining the functional integrity of skeletal muscle.

Key words: {alpha}7 integrin, Dystrophin, Transgenic mice, Double knockout, Skeletal muscle, Muscular dystrophy


Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


This article has been cited by other articles:


Home page
 DevelopmentHome page
F. J. Conti, S. J. Monkley, M. R. Wood, D. R. Critchley, and U. Muller
Talin 1 and 2 are required for myoblast fusion, sarcomere assembly and the maintenance of myotendinous junctions
Development, November 1, 2009; 136(21): 3597 - 3606.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
J. V. Welser, J. E. Rooney, N. C. Cohen, P. B. Gurpur, C. A. Singer, R. A. Evans, B. A. Haines, and D. J. Burkin
Myotendinous Junction Defects and Reduced Force Transmission in Mice that Lack {alpha}7 Integrin and Utrophin
Am. J. Pathol., October 1, 2009; 175(4): 1545 - 1554.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
J. E. Rooney, P. B. Gurpur, and D. J. Burkin
Laminin-111 protein therapy prevents muscle disease in the mdx mouse model for Duchenne muscular dystrophy
PNAS, May 12, 2009; 106(19): 7991 - 7996.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
D. Li, C. Long, Y. Yue, and D. Duan
Sub-physiological sarcoglycan expression contributes to compensatory muscle protection in mdx mice
Hum. Mol. Genet., April 1, 2009; 18(7): 1209 - 1220.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
A. K. Peter, C. Y. Ko, M. H. Kim, N. Hsu, N. Ouchi, S. Rhie, Y. Izumiya, L. Zeng, K. Walsh, and R. H. Crosbie
Myogenic Akt signaling upregulates the utrophin-glycoprotein complex and promotes sarcolemma stability in muscular dystrophy
Hum. Mol. Genet., January 15, 2009; 18(2): 318 - 327.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
J. E. Rooney, P. B. Gurpur, Z. Yablonka-Reuveni, and D. J. Burkin
Laminin-111 Restores Regenerative Capacity in a Mouse Model for {alpha}7 Integrin Congenital Myopathy
Am. J. Pathol., January 1, 2009; 174(1): 256 - 264.
[Abstract] [Full Text] [PDF]

Home page
 DevelopmentHome page
F. J. Conti, A. Felder, S. Monkley, M. Schwander, M. R. Wood, R. Lieber, D. Critchley, and U. Muller
Progressive myopathy and defects in the maintenance of myotendinous junctions in mice that lack talin 1 in skeletal muscle
Development, June 1, 2008; 135(11): 2043 - 2053.
[Abstract] [Full Text] [PDF]

Home page
 Circ. Res.Home page
L. Elsherif, M. S. Huang, S.-Y. Shai, Y. Yang, R. Y. Li, J. Chun, M. A. Mekany, A. L. Chu, S. J. Kaufman, and R. S. Ross
Combined Deficiency of Dystrophin and {beta}1 Integrin in the Cardiac Myocyte Causes Myocardial Dysfunction, Fibrosis and Calcification
Circ. Res., May 9, 2008; 102(9): 1109 - 1117.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
H.-V. Wang, L.-W. Chang, K. Brixius, S. A. Wickstrom, E. Montanez, I. Thievessen, M. Schwander, U. Muller, W. Bloch, U. Mayer, et al.
Integrin-linked kinase stabilizes myotendinous junctions and protects muscle from stress-induced damage
J. Cell Biol., March 5, 2008; 180(5): 1037 - 1049.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Cell Physiol.Home page
J. Liu, D. J. Burkin, and S. J. Kaufman
Increasing {alpha}7{beta}1-integrin promotes muscle cell proliferation, adhesion, and resistance to apoptosis without changing gene expression
Am J Physiol Cell Physiol, February 1, 2008; 294(2): C627 - C640.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
P. Sharma, T. Tran, G. L. Stelmack, K. McNeill, R. Gosens, M. M. Mutawe, H. Unruh, W. T. Gerthoffer, and A. J. Halayko
Expression of the dystrophin-glycoprotein complex is a marker for human airway smooth muscle phenotype maturation
Am J Physiol Lung Cell Mol Physiol, January 1, 2008; 294(1): L57 - L68.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
A. L. Gheyara, A. Vallejo-Illarramendi, K. Zang, L. Mei, R. St.-Arnaud, S. Dedhar, and L. F. Reichardt
Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency
Am. J. Pathol., December 1, 2007; 171(6): 1966 - 1977.
[Abstract] [Full Text] [PDF]

Home page
 Circ. Res.Home page
J. V. Welser, N. Lange, C. A. Singer, M. Elorza, P. Scowen, K. D. Keef, W. T. Gerthoffer, and D. J. Burkin
Loss of the {alpha}7 Integrin Promotes Extracellular Signal-Regulated Kinase Activation and Altered Vascular Remodeling
Circ. Res., September 28, 2007; 101(7): 672 - 681.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
D. J. Milner and S. J. Kaufman
{alpha}7{beta}1 Integrin Does Not Alleviate Disease in a Mouse Model of Limb Girdle Muscular Dystrophy Type 2F
Am. J. Pathol., February 1, 2007; 170(2): 609 - 619.
[Abstract] [Full Text] [PDF]


© The Company of Biologists Ltd 2006