help button home button Am J Pathol Angiogenesis Meeting
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS

This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Purchase Article
Right arrow View Shopping Cart
Services
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 Ozawa, R.
Right arrow Articles by Nishino, I.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Ozawa, R.
Right arrow Articles by Nishino, I.
(American Journal of Pathology. 2006;168:907-917.)
© 2006 American Society for Investigative Pathology

Emerin-Lacking Mice Show Minimal Motor and Cardiac Dysfunctions with Nuclear-Associated Vacuoles

Ritsuko Ozawa, Yukiko K. Hayashi, Megumu Ogawa, Rumi Kurokawa, Hiroshi Matsumoto, Satoru Noguchi, Ikuya Nonaka and Ichizo Nishino

From the Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan

Emery-Dreifuss muscular dystrophy is an inherited muscular disorder clinically characterized by slowly progressive weakness affecting humero-peroneal muscles, early joint contractures, and cardiomyopathy with conduction block. The X-linked recessive form is caused by mutation in the EMD gene encoding an integral protein of the inner nuclear membrane, emerin. In this study, mutant mice lacking emerin were produced by insertion of a neomycin resistance gene into exon 6 of the coding gene. Tissues taken from mutant mice lacked emerin. The mutant mice displayed a normal growth rate indistinguishable from their littermates and were fertile. No marked muscle weakness or joint abnormalities were observed; however, rotarod test revealed altered motor coordination. Electrocardiography showed mild prolongation of atrioventricular conduction time in emerin-lacking male mice older than 40 weeks of age. Electron microscopic analysis of skeletal and cardiac muscles from emerin-lacking mice revealed small vacuoles, which mostly bordered the myonuclei. Our results suggest that emerin deficiency causes minimal motor and cardiac dysfunctions in mice with a structural fragility of myonuclei.




This article has been cited by other articles:


Home page
 Circ. Res.Home page
J. M. Holaska
Emerin and the Nuclear Lamina in Muscle and Cardiac Disease
Circ. Res., July 3, 2008; 103(1): 16 - 23.
[Abstract] [Full Text] [PDF]

Home page
 J BiochemHome page
Y. Tsuchiya
Till Disassembly Do Us Part: A Happy Marriage of Nuclear Envelope and Chromatin
J. Biochem., February 1, 2008; 143(2): 155 - 161.
[Abstract] [Full Text] [PDF]

Home page
 J. Virol.Home page
N. Leach, S. L. Bjerke, D. K. Christensen, J. M. Bouchard, F. Mou, R. Park, J. Baines, T. Haraguchi, and R. J. Roller
Emerin Is Hyperphosphorylated and Redistributed in Herpes Simplex Virus Type 1-Infected Cells in a Manner Dependent on both UL34 and US3
J. Virol., October 1, 2007; 81(19): 10792 - 10803.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
A. Muchir, P. Pavlidis, G. Bonne, Y. K. Hayashi, and H. J. Worman
Activation of MAPK in hearts of EMD null mice: similarities between mouse models of X-linked and autosomal dominant Emery Dreifuss muscular dystrophy
Hum. Mol. Genet., August 1, 2007; 16(15): 1884 - 1895.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2006 by the American Society for Investigative Pathology.