Anti-Dystrophin Polyclonal Antibody
Product Specifications
CAS Number
9007-83-4
Gene Name
Dystrophin
Gene Aliases
BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; dystrophin; MRX85; truncated dystrophin.
Gene ID
1756
Swiss Prot
P11532
Reactivity
Human|Mouse
Immunogen
A synthetic peptide corresponding to the intra domain 410-450aa of human Dystrophin protein. This sequence is identical to mouse and Pan troglodytes and other species.
Target
The dystrophin gene is the largest gene found in nature. The gene was identified throμ gh 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. IHC staining of normal muscle tissue results in clear labeling confined to the periphery (plasma membrane) of normal muscle fibers. The product exhibits wide interspecies cross-reactivity.
Clonality
Polyclonal
Type
Polyclonal Antibody
Applications
Enzyme-linked immunosorbent assay|Flow cytometry|Immunohistochemistry|Immunoprecipitation|Western blot
Purification
Affinity Purified
Assay Protocol
Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions
Reconstitution & Storage Instructions
Western Blotting/Immunoblotting (WB/IB) Protocol
Western Blotting/Immunoblotting (WB/IB) Protocol
Immunohistochemistry (IHC) Protocol
Immunohistochemistry (IHC) Protocol
Immunocytochemistry (ICC) Protocol
Immunocytochemistry (ICC) Protocol
Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol
Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol
Blocking Peptide Competition Protocol (BPCP)
Blocking Peptide Competition Protocol (BPCP)
Immunoprecipitation (IP) Protocol
Immunoprecipitation (IP) Protocol
Antibody Array (AA) Protocol
Antibody Array (AA) Protocol
Concentration
100ug/200ul
Format
Liquid. This affinity purified antibody is supplied in sterile Phosphatebuffered saline (pH7.2) containing antibody stabilizer
Reconstitution
The antibodies are stable for 24 months from date of receipt when stored at -20C to -70C. The antibodies can be stored at 2C-8C for three month without detectable loss of activity. Avoid repeated freezing-thawing cycles.
Molecular Weight
>110
Fragment
Rabbit IgG
Specificity
DMD
NCBI Gene Symbol
DMD
Host or Source
Rabbit
Gene Name URL
DMD
Frequently Asked Questions
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