Click here to close
Hello! We notice that you are using Internet Explorer, which is not supported by Xenbase and may cause the site to display incorrectly.
We suggest using a current version of Chrome,
FireFox, or Safari.
Biochem J
1992 Dec 01;288 ( Pt 2):663-8. doi: 10.1042/bj2880663.
Show Gene links
Show Anatomy links
Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections.
Nguyen TM, Ginjaar IB, van Ommen GJ, Morris GE.
???displayArticle.abstract???
A group of 44 monoclonal antibodies (mAbs) raised against the central helical rod (25 mAbs) and C-terminal (19 mAbs) regions of dystrophin were prepared using trpE recombinant fusion proteins as immunogens. Some mAbs cross-react with the structurally related proteins, alpha-actinin and utrophin. Epitope mapping revealed uneven distribution of mAb-binding sites, no mAbs being produced against the C-terminal end of the helical fragment or the cysteine-rich region of the C-terminal dystrophin fragment. The failure of these large regions of the recombinant immunogens to elicit anti-dystrophin antibodies may be because of their inability to fold into the correct dystrophin-like conformation. The mAbs were selected for their ability to recognize 427 kDa dystrophin on Western blots after SDS/PAGE, and/or for immunostaining of the membrane in frozen muscle sections. Although some mAbs obtained by Western-blot screening failed to bind native dystrophin in frozen muscle sections, successful binding could be obtained after SDS or urea treatment of the tissue section to expose the epitopes. This increases the range of mAbs available for detection of dystrophin deletions in muscular dystrophy and evaluation of myoblast therapy.
Bar,
A novel product of the Duchenne muscular dystrophy gene which greatly differs from the known isoforms in its structure and tissue distribution.
1990, Pubmed
Bar,
A novel product of the Duchenne muscular dystrophy gene which greatly differs from the known isoforms in its structure and tissue distribution.
1990,
Pubmed Beggs,
Exploring the molecular basis for variability among patients with Becker muscular dystrophy: dystrophin gene and protein studies.
1991,
Pubmed Blake,
Characterization of a 4.8kb transcript from the Duchenne muscular dystrophy locus expressed in Schwannoma cells.
1992,
Pubmed Dieckmann,
Assembly of the mitochondrial membrane system. CBP6, a yeast nuclear gene necessary for synthesis of cytochrome b.
1985,
Pubmed Ellis,
Specificity of dystrophin analysis improved with monoclonal antibodies.
1990,
Pubmed England,
Very mild muscular dystrophy associated with the deletion of 46% of dystrophin.
1990,
Pubmed Feener,
Alternative splicing of human dystrophin mRNA generates isoforms at the carboxy terminus.
1989,
Pubmed Ginjaar,
Immunohistochemical studies show truncated dystrophins in the myotubes of three fetuses at risk for Duchenne muscular dystrophy.
1991,
Pubmed Ginjaar,
Fetal dystrophin to diagnose carrier status.
1991,
Pubmed Ginjaar,
Immunological study of dystrophin in Duchenne fetus.
1989,
Pubmed Gussoni,
Normal dystrophin transcripts detected in Duchenne muscular dystrophy patients after myoblast transplantation.
1992,
Pubmed Helliwell,
A truncated dystrophin lacking the C-terminal domains is localized at the muscle membrane.
1992,
Pubmed Hoffman,
Characterization of dystrophin in muscle-biopsy specimens from patients with Duchenne's or Becker's muscular dystrophy.
1988,
Pubmed Hoffman,
Detection of a specific isoform of alpha-actinin with antisera directed against dystrophin.
1989,
Pubmed Hoffman,
Dystrophin: the protein product of the Duchenne muscular dystrophy locus.
1987,
Pubmed Hugnot,
Distal transcript of the dystrophin gene initiated from an alternative first exon and encoding a 75-kDa protein widely distributed in nonmuscle tissues.
1992,
Pubmed Koenig,
The molecular basis for Duchenne versus Becker muscular dystrophy: correlation of severity with type of deletion.
1989,
Pubmed Koenig,
The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein.
1988,
Pubmed Koenig,
Detailed analysis of the repeat domain of dystrophin reveals four potential hinge segments that may confer flexibility.
1990,
Pubmed Laemmli,
Cleavage of structural proteins during the assembly of the head of bacteriophage T4.
1970,
Pubmed Lederfein,
A 71-kilodalton protein is a major product of the Duchenne muscular dystrophy gene in brain and other nonmuscle tissues.
1992,
Pubmed Lemaire,
The chicken dystrophin cDNA: striking conservation of the C-terminal coding and 3' untranslated regions between man and chicken.
1988,
Pubmed Love,
An autosomal transcript in skeletal muscle with homology to dystrophin.
1989,
Pubmed Morris,
Monoclonal antibody studies suggest a catalytic site at the interface between domains in creatine kinase.
1990,
Pubmed Nguyen,
Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines.
1991,
Pubmed
,
Xenbase Nguyen,
Monoclonal antibody evidence for structural similarities between the central rod regions of actinin and dystrophin.
1990,
Pubmed Nguyen thi Man,
Structural changes in the C-terminal region of human brain creatine kinase studied with monoclonal antibodies.
1991,
Pubmed Nguyen thi Man,
Monoclonal antibodies against defined regions of the muscular dystrophy protein, dystrophin.
1990,
Pubmed Nicholson,
Heterogeneity of dystrophin expression in patients with Duchenne and Becker muscular dystrophy.
1990,
Pubmed Récan,
Are cysteine-rich and COOH-terminal domains of dystrophin critical for sarcolemmal localization?
1992,
Pubmed Sedgwick,
Rapid mapping by transposon mutagenesis of epitopes on the muscular dystrophy protein, dystrophin.
1991,
Pubmed Simmerman,
Secondary structure of detergent-solubilized phospholamban, a phosphorylatable, oligomeric protein of cardiac sarcoplasmic reticulum.
1989,
Pubmed Tanaka,
Expression of a dystrophin-related protein associated with the skeletal muscle cell membrane.
1991,
Pubmed Walsh,
Tissue-specific isoforms of dystrophin.
1989,
Pubmed
