NDLI: Overexpression of the Cytotoxic T Cell (CT) Carbohydrate Inhibits Muscular Dystrophy in the dyW Mouse Model of Congenital Muscular Dystrophy 1A

Content Provider	PubMed Central
Author	Xu, Rui Chandrasekharan, Kumaran Yoon, Jung Hae Camboni, Marybeth Martin, Paul T.
Copyright Year	2007
Abstract	A number of recent studies have demonstrated therapeutic effects of transgenes on the development of muscle pathology in the mdx mouse model for Duchenne muscular dystrophy, but none have been shown also to be effective in mouse models for laminin α2-deficient congenital muscular dystrophy (MDC1A). Here, we show that overexpression of the cytotoxic T cell (CT) GalNAc transferase (Galgt2) is effective in inhibiting the development of muscle pathology in the dyW mouse model of MDC1A, much as we had previously shown in mdx animals. Embryonic overexpression of Galgt2 in skeletal muscles using transgenic mice or postnatal overexpression using adeno-associated virus both reduced the extent of muscle pathology in dyW/dyW skeletal muscle. As with mdx mice, embryonic overexpression of the Galgt2 transgene in dyW/dyW myofibers inhibited muscle growth, whereas postnatal overexpression did not. Both embryonic and postnatal overexpression of Galgt2 in dyW/dyW muscle increased the expression of agrin, a protein that, in recombinant form, has been shown to ameliorate disease, whereas laminin α1, another disease modifier, was not expressed. Galgt2 over-expression also stimulated the glycosylation of a glycolipid with the CT carbohydrate, and glycolipids accounted for most of the CT-reactive material in postnatal overexpression experiments. These experiments demonstrate that Galgt2 overexpression is effective in altering disease progression in skeletal muscles of dyW mice and should be considered as a therapeutic target in MDC1A.
Related Links	http://dx.doi.org/10.2353/ajpath.2007.060927
Ending Page	199
Page Count	19
Starting Page	181
File Format	PDF
ISSN	00029440
e-ISSN	15252191
Journal	The American Journal of Pathology
Issue Number	1
Volume Number	171
Language	English
Publisher	American Society for Investigative Pathology
Publisher Date	2007-07-01
Access Restriction	Open
Rights Holder	American Society for Investigative Pathology
Subject Keyword	Pathology and Forensic Medicine Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Pathology and Forensic Medicine

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