DMD recombinant monoclonal antibody, clone 4B10
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Specification
Product Description
Rabbit recombinant monoclonal antibody raised against human DMD.
Antibody Species
Rabbit
Immunogen
Original antibody is raised against recombinant protein corresponding to full length human DMD.
Reactivity
Human
Form
Liquid
Purification
Affinity-chromatography
Isotype
IgG
Recommend Usage
ELISA
Immunohistochemistry (1:50-1:200)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS, pH7.4 (150mM NaCl, 50% glycerol and 0.02% sodium azide)
Storage Instruction
Store at -20 °C or -80 °C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Immunohistochemistry
Immunohistochemistry image of DMD recombinant monoclonal antibody, clone 4B10 diluted at 1:100 and staining in paraffin-embedded human heart tissue performed on a Leica BondTM system.Enzyme-linked Immunoabsorbent Assay
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Gene Info — DMD
Entrez GeneID
1756Protein Accession#
P11532Gene Name
DMD
Gene Alias
BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272
Gene Description
dystrophin
Gene Ontology
HyperlinkGene Summary
The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq
Other Designations
Duchenne muscular dystrophy protein|OTTHUMP00000023117|OTTHUMP00000023124|OTTHUMP00000023125|OTTHUMP00000023126|muscular dystrophy, Duchenne and Becker types
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Interactome
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Pathway
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Disease
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