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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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