MABT827-AF555

Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor^™ 555 Conjugate

clone 2C6 (MANDYS106), from mouse, ALEXA FLUOR^™ 555

• Synonym(s): Dystrophin
• UNSPSC Code: 12352203
• eCl@ss: 32160702

Properties

• biological source: mouse
• Quality Level: 100
• conjugate: ALEXA FLUOR^™ 555
• antibody form: purified immunoglobulin
• antibody product type: primary antibodies
• clone: 2C6 (MANDYS106), monoclonal
• species reactivity: human
• technique(s): immunofluorescence: suitable
• isotype: IgG2aκ
• NCBI accession no.: NP_000100
• UniProt accession no.: P11532
• shipped in: ambient
• target post-translational modification: unmodified
• Gene Information: human ... DMD(1756)

Description

General description

Dystrophin (UniProt P11532) is encoded by the DMD (also known as BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85) gene (Gene ID 1756) in human. Dystrophin is localized to the inner part of the muscle fiber cell membrane (sarcolemma), where it forms the dystrophin-associated glycoprotein complex (DGC) that links the extracellular matrix to the actin cytoskeleton. Dystrophin plays an important role in stabilizing the muscle fibre against the mechanical forces of muscle contraction by providing a shock-absorbing connection between the cytoskeleton and the extracellular matrix. Duchenne muscular dystrophy (DMD) is caused by gene mutations that disrupt the open reading frame (ORF) and prevent the full translation of dystrophin. ORF restoration by exon skipping using antisense oligonucleotides targeted to splicing elements are designed to transform the DMD phenotype to that of the milder disorder, Becker muscular dystrophy (BMD), typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin.

Application

The unconjugated antibody (Cat. No. MABT827) and Alexa Fluor^™ 488 conjugate (Cat. No. MABT827-AF488) are also available for Immunohistochemistry, Immunofluorescence, and Western Blotting applications.

This mouse monoclonal Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor^™ 555 Conjugate, Cat. No. MABT827-AF555 is validated for use in Immunofluorescence for the detection of Dystrophin.

Quality

Evaluated by Immunofluorescence in human skeletal muscle tissue sections.

Immunofluorescence Analysis: A 1:100 dilution of this antibody detected dystrophin in human skeletal muscle tissue sections.

Target description

~427 kDa observed.

Physical form

Purified mouse IgG2a Alexa Fluor^™ 555 conjugate in buffer containing PBS, 1.5% BSA with 0.05% sodium azide

Analysis Note

Control
The unconjugated antibody (Cat. No. MABT827) and Alexa Fluor^™ 488 conjugate (Cat. No. MABT827-AF488) are also available for Immunohistochemistry

Immunofluorescence

and Western Blotting applications.

Other Notes

Concentration: Please refer to lot specific datasheet.

Legal Information

ALEXA FLUOR is a trademark of Life Technologies

Safety Information

• Storage Class: 12 - Non Combustible Liquids
• wgk_germany: WGK 2
• flash_point_f: Not applicable
• flash_point_c: Not applicable