Muscular dystrophies involving the dystrophin-glycoprotein complex: an overview of current mouse models
(2002) In Current Opinion in Genetics & Development 12(3). p.349-361- Abstract
- The dystrophin-glycoprotein complex (DGC) is a multisubunit complex that connects the cytoskeleton of a muscle fiber to its surrounding extracellular matrix. Mutations in the DGC disrupt the complex and lead to muscular dystrophy. There are a few naturally occurring animal models of DGC-associated muscular dystrophy (e.g. the dystrophin-deficient mdx mouse, dystrophic golden retriever dog, HFMD cat and the delta-sarcoglycan-deficient BIO 14.6 cardiomyopathic hamster) that share common genetic protein abnormalities similar to those of the human disease. However, the naturally occurring animal models only partially resemble human disease. In addition, no naturally occurring mouse models associated with loss of other DGC components are... (More)
- The dystrophin-glycoprotein complex (DGC) is a multisubunit complex that connects the cytoskeleton of a muscle fiber to its surrounding extracellular matrix. Mutations in the DGC disrupt the complex and lead to muscular dystrophy. There are a few naturally occurring animal models of DGC-associated muscular dystrophy (e.g. the dystrophin-deficient mdx mouse, dystrophic golden retriever dog, HFMD cat and the delta-sarcoglycan-deficient BIO 14.6 cardiomyopathic hamster) that share common genetic protein abnormalities similar to those of the human disease. However, the naturally occurring animal models only partially resemble human disease. In addition, no naturally occurring mouse models associated with loss of other DGC components are available. This has encouraged the generation of genetically engineered mouse models for DGC-linked muscular dystrophy. Not only have analyses of these mice led to a significant improvement in our understanding of the pathogenetic mechanisms for the development of muscular dystrophy, but they will also be immensely valuable tools for the development of novel therapeutic approaches for these incapacitating diseases. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/1125380
- author
- Durbeej-Hjalt, Madeleine LU and Campbell, Kevin P
- publishing date
- 2002
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Current Opinion in Genetics & Development
- volume
- 12
- issue
- 3
- pages
- 349 - 361
- publisher
- Elsevier
- external identifiers
-
- pmid:12076680
- scopus:0036591684
- ISSN
- 1879-0380
- DOI
- 10.1016/S0959-437X(02)00309-X
- language
- English
- LU publication?
- no
- id
- d4b4e5b2-c39b-438c-94af-0a3cfd413ec4 (old id 1125380)
- date added to LUP
- 2016-04-01 15:18:15
- date last changed
- 2022-03-22 03:42:11
@article{d4b4e5b2-c39b-438c-94af-0a3cfd413ec4,
abstract = {{The dystrophin-glycoprotein complex (DGC) is a multisubunit complex that connects the cytoskeleton of a muscle fiber to its surrounding extracellular matrix. Mutations in the DGC disrupt the complex and lead to muscular dystrophy. There are a few naturally occurring animal models of DGC-associated muscular dystrophy (e.g. the dystrophin-deficient mdx mouse, dystrophic golden retriever dog, HFMD cat and the delta-sarcoglycan-deficient BIO 14.6 cardiomyopathic hamster) that share common genetic protein abnormalities similar to those of the human disease. However, the naturally occurring animal models only partially resemble human disease. In addition, no naturally occurring mouse models associated with loss of other DGC components are available. This has encouraged the generation of genetically engineered mouse models for DGC-linked muscular dystrophy. Not only have analyses of these mice led to a significant improvement in our understanding of the pathogenetic mechanisms for the development of muscular dystrophy, but they will also be immensely valuable tools for the development of novel therapeutic approaches for these incapacitating diseases.}},
author = {{Durbeej-Hjalt, Madeleine and Campbell, Kevin P}},
issn = {{1879-0380}},
language = {{eng}},
number = {{3}},
pages = {{349--361}},
publisher = {{Elsevier}},
series = {{Current Opinion in Genetics & Development}},
title = {{Muscular dystrophies involving the dystrophin-glycoprotein complex: an overview of current mouse models}},
url = {{http://dx.doi.org/10.1016/S0959-437X(02)00309-X}},
doi = {{10.1016/S0959-437X(02)00309-X}},
volume = {{12}},
year = {{2002}},
}