| Laminin alpha1 chain reduces muscular dystrophy in laminin alpha2 chain deficient mice. | |
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MedLine Citation:
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PMID: 15213105 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Laminin (LN) alpha2 chain deficiency in humans and mice leads to severe forms of congenital muscular dystrophy (CMD). Here, we investigated whether LNalpha1 chain in mice can compensate for the absence of LNalpha2 chain and prevent the development of muscular dystrophy. We generated mice expressing a LNalpha1 chain transgene in skeletal muscle of LNalpha2 chain deficient mice. LNalpha1 is not normally expressed in muscle, but the transgenically produced LNalpha1 chain was incorporated into muscle basement membranes, and normalized the compensatory changes of expression of certain other laminin chains (alpha4, beta2). In 4-month-old mice, LNalpha1 chain could fully prevent the development of muscular dystrophy in several muscles, and partially in others. The LNalpha1 chain transgene not only reversed the appearance of histopathological features of the disease to a remarkable degree, but also greatly improved health and longevity of the mice. Correction of LNalpha2 chain deficiency by LNalpha1 chain may serve as a paradigm for gene therapy of CMD in patients. |
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Authors:
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Kinga Gawlik; Yuko Miyagoe-Suzuki; Peter Ekblom; Shin'ichi Takeda; Madeleine Durbeej |
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Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't Date: 2004-06-22 |
Journal Detail:
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Title: Human molecular genetics Volume: 13 ISSN: 0964-6906 ISO Abbreviation: Hum. Mol. Genet. Publication Date: 2004 Aug |
Date Detail:
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Created Date: 2004-07-20 Completed Date: 2005-02-25 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9208958 Medline TA: Hum Mol Genet Country: England |
Other Details:
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Languages: eng Pagination: 1775-84 Citation Subset: IM |
Affiliation:
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Department of Cell and Molecular Biology, Section of Cell and Developmental Biology, University of Lund, Sweden. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Basement Membrane / metabolism DNA Primers Fluorescent Antibody Technique Gene Expression Regulation* Gene Therapy / methods Genotype Laminin / deficiency*, genetics* Mice Mice, Transgenic Microscopy, Electron, Transmission Muscle, Skeletal / metabolism, ultrastructure Muscular Dystrophies / genetics*, metabolism*, pathology Reverse Transcriptase Polymerase Chain Reaction Transgenes / genetics |
| Chemical | |
Reg. No./Substance:
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0/DNA Primers; 0/Laminin; 0/laminin alpha 2; 151186-83-3/laminin A |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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