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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
6
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pubmed:dateCreated |
1995-7-18
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pubmed:abstractText |
In skeletal muscle, dystrophin exists in a large oligomeric complex tightly associated with several novel sarcolemmal proteins, including the 50-kDa transmembrane glycoprotein called adhalin. The dystrophin-glycoprotein complex links the subsarcolemmal actin cytoskeleton to the basal lamina component laminin, thus providing stability to the sarcolemma. Disturbance of this linkage due to the absence of dystrophin plays a crucial role in the molecular pathogenesis of muscle fiber necrosis in Duchenne muscular dystrophy. Severe childhood autosomal recessive muscular dystrophy (SCARMD) is similar to Duchenne muscular dystrophy in phenotype but is characterized by the deficiency of adhalin. At present, the status of the link between the dystrophin-glycoprotein complex and laminin is unclear in SCARMD.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Jun
|
pubmed:issn |
0023-6837
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
72
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pubmed:owner |
NLM
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pubmed:authorsComplete |
N
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pubmed:pagination |
715-22
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:7783429-Adolescent,
pubmed-meshheading:7783429-Adult,
pubmed-meshheading:7783429-Child,
pubmed-meshheading:7783429-Cytoskeletal Proteins,
pubmed-meshheading:7783429-Female,
pubmed-meshheading:7783429-Genes, Recessive,
pubmed-meshheading:7783429-Humans,
pubmed-meshheading:7783429-Immunohistochemistry,
pubmed-meshheading:7783429-Laminin,
pubmed-meshheading:7783429-Male,
pubmed-meshheading:7783429-Membrane Glycoproteins,
pubmed-meshheading:7783429-Microscopy, Confocal,
pubmed-meshheading:7783429-Muscle, Skeletal,
pubmed-meshheading:7783429-Muscular Dystrophies,
pubmed-meshheading:7783429-Sarcoglycans
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pubmed:year |
1995
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pubmed:articleTitle |
Laminin abnormality in severe childhood autosomal recessive muscular dystrophy.
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pubmed:affiliation |
Department of Neurology and Neuroscience, Teikyo University School of Medicine, Tokyo, Japan.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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