DMD, Mouse, Clone: DYS-48, Abnova™

Mouse monoclonal antibody raised against full-length recombinant DMD.

Supplier:  Abnova Corporation MAB1453

Catalog No. 89-110-437

Description

The dystrophin gene is the largest gene found in nature, measuring 2.4Mb. 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 3500 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

Specifications

• Antigen: DMD
• Classification: Monoclonal
• Conjugate: Unconjugated
• Dilution: Western Blot (1-2 ug/mL) Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) (2-4 ug/mL) The optimal working dilution should be determined by the end user.
• Gene: DMD
• Gene Symbols: DMD
• Immunogen: Recombinant protein corresponding to full length human DMD.
• Quantity: 100 μg
• Primary or Secondary: Primary
• Target Species: Chicken, Human, Mouse, Rat
• Form: Lyophilized

• Applications: Immunohistochemistry (Frozen), Western Blot
• Clone: MANDYS8
• Description: Mouse monoclonal antibody raised against full length recombinant DMD.
• Formulation: Lyophilized from 1.2% sodium acetate (2mg BSA, 0.01mg sodium azide)
• Gene Alias: BMD/CMD3B/DXS142/DXS164/DXS206/DXS230/DXS239/DXS268/DXS269/DXS270/DXS272
• Host Species: Mouse
• Purification Method: Affinity purification
• Regulatory Status: RUO
• Gene ID (Entrez): 1756
• Content And Storage: Store at -20°C on dry atmosphere. After reconstitution with 1mL of 1.2% sodium acetate or neutral PBS to a concentration of 100μg/mL store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
• Isotype: IgG2b