Mouse monoclonal antibody raised against full length recombinant DMD.
Immunogen:
Recombinant protein corresponding to full length human DMD.
Host:
Mouse
Theoretical MW (kDa):
426.6
Reactivity:
Chicken, Human, Mouse, Rat
Form:
Lyophilized
Purification:
Affinity purification
Isotype:
IgG2b
Quality Control Testing:
Antibody Reactive Against Recombinant Protein.
Recommend Usage:
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.
Store at -20°C on dry atmosphere. After reconstitution with 1 mL of 1.2% sodium acetate or neutral PBS and concentration will be 100 ug/mL, store at -20°C or lower. 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.
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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