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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
2
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pubmed:dateCreated |
1994-5-16
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pubmed:abstractText |
Dystrophin is a muscle cytoskeletal protein with a molecular mass (MM) of approximately 420 kDa and an isoelectric point (pI) of approximately 5.5, which is abnormal in size and/or abundance in Becker muscular dystrophy (BMD). We investigated the abnormality of dystrophin molecule in muscles biopsied from 23 BMD patients using the two-dimensional gel electrophoresis (TDGE). We found 7 protein spots which reacted specifically with the monoclonal anti-dystrophin antibody (mAb) A1C raised against N-terminal domain of the normal dystrophin. These spots were focused on the two-dimensional gel at the same position as the normal dystrophin (#1), at the position with MM approximately 480 kDa/pI approximately 5.35 (#2), the position with MM approximately 400-330 kDa/pI approximately 5.51-5.47 (#3), the position with MM approximately 300 kDa/pI approximately 5.4 (#4), the position with MM approximately 235-250 kDa/pI approximately 5.53-5.5 (#5), the position with MM approximately 165 kDa/pI approximately 6.0 (#6), and the position with MM approximately 160 kDa/pI approximately 5.75 (#7). These spots were classified into five patterns in individuals, that is, #1 alone in 3 patients, #3 alone in 1, the combination of #3 and 5 in 17, the combination of #1, 3 and 5 in 1 and the combination of #1, 2, 4, 6 and 7 in 1. The combination of #3 and 5 was observed in 17 of 23 patients (75%).(ABSTRACT TRUNCATED AT 250 WORDS)
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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 |
Feb
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pubmed:issn |
0022-510X
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
121
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
183-9
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:8158213-Adolescent,
pubmed-meshheading:8158213-Adult,
pubmed-meshheading:8158213-Antibodies, Monoclonal,
pubmed-meshheading:8158213-Blotting, Western,
pubmed-meshheading:8158213-Child,
pubmed-meshheading:8158213-Dystrophin,
pubmed-meshheading:8158213-Electrophoresis, Gel, Two-Dimensional,
pubmed-meshheading:8158213-Female,
pubmed-meshheading:8158213-Humans,
pubmed-meshheading:8158213-Isoelectric Focusing,
pubmed-meshheading:8158213-Male,
pubmed-meshheading:8158213-Middle Aged,
pubmed-meshheading:8158213-Molecular Weight,
pubmed-meshheading:8158213-Muscle Proteins,
pubmed-meshheading:8158213-Muscular Diseases,
pubmed-meshheading:8158213-Muscular Dystrophies
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pubmed:year |
1994
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pubmed:articleTitle |
Multiplicity of abnormal dystrophin in Becker muscular dystrophy. A Becker muscular dystrophy gene frequently produced two smaller sizes of dystrophin.
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pubmed:affiliation |
Department of Molecular and Cellular Neurobiology, Tokyo Metropolitan Institute for Neuroscience, Japan.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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