DMD Antibody - C-terminal region
Product Specifications
CAS Number
9007-83-4
Gene Name
Dystrophin
Gene Aliases
BMD, CMD3B, MRX85, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272
Gene ID
1756
Swiss Prot
P11532-8
Accession Number
NP_000100.2
Reactivity
Human
Immunogen
The immunogen is a synthetic peptide directed towards the C terminal region of human DMD
Target
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.
Clonality
Polyclonal
Type
Polyclonal Antibody
Sequence
SQTSDSMGEEDLLSPPQDTSTGLEEVMEQLNNSFPSSRGRNTPGKPMRED
Applications
WB
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
0.5 mg/ml
Format
Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Reconstitution
For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
Molecular Weight
66 kDa
Protein Length
604
NCBI Gene Symbol
DMD
Host or Source
Rabbit
Protein Name
Dystrophin
Gene Name URL
DMD
Nucleotide Accession Number
NM_000109.3
Frequently Asked Questions
More Discoveries
Explore Other Products
Browse additional items from our catalog