Rabbit anti-Human, Mouse Dystrophin Polyclonal Antibody | anti-DMD antibody

Rabbit anti Dystrophin Polyclonal Antibody

Cat. Num. AAA460575

• Gene Names: DMD; BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
• Reactivity: Human, Mouse
• Applications: Western Blot, ELISA, Immunoprecipitation, Immunohistochemistry
• Purity: The Rabbit IgG is purified by Epitope Affinity purification.
• Synonyms: Dystrophin; Polyclonal Antibody; Rabbit anti Dystrophin Polyclonal Antibody; DMD; Dp140bc; anti-DMD antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: This antibody recognizes dystrophine isoforms. It reacts with human and mouse origins. The other species are not tested.
• Purity/Purification: The Rabbit IgG is purified by Epitope Affinity purification.
• Form/Format: The affinity purified antibody is supplied in sterile phosphate buffered saline (pH 7.2) containing antibody stabilizer.

• Applicable Applications for anti-DMD antibody: Western Blot (WB) , ELISA (EIA), Immunoprecipitation (IP), Immunohistochemistry (IHC)
• Application Notes: Western Blot 0.1-1 ug/ml; ELISA 0.01- 0.1 ug/ml; Immunoprecipitation 2-5 ug/ml; Immunohistochemistry 2-10 ug/ml; ; Optimal dilutions should be determined by researchers for the specific applications.
• Antigen Preparation: 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.
• Positive Control: Kidney Tissue
• Preparation and Storage: The antibodies are stable for 12 months from date of receipt when stored at -20°C to -70°C. The antibodies can be stored at 2-8°C for three months without detectable loss of activity. Avoid repeated freezing-thawing cycles.

Western Blot (WB)

(Western Blot: The tissue lysate derived from mouse skeletal muscle was immuno-blotted by Rabbit anti Dystrophin (MBS460575) at 1:500. Multiple bands between 71kDa-250 kDa were observed.)

Immunohistochemistry (IHC)

(Immunohistochemistry (IHC) Mouse skeletal muscle stained with Rabbit anti - Dystrophin antibody, (MBS460575) at 1:200 for 10 min @ RT. Staining of formalin- fixed tissue requires boiling tissue sections in 10 mM Citrate Buffer, pH 6.0 for 10 min followed by cooling at RT for 20 min.)

Related Product Information for anti-DMD antibody

The dystrophin gene is the largest gene found in nature. 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. 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.

References

Sarkis, J., et al. Spectrin-like repeats 11-15 of human dystrophin show adaptations to a lipidic environment. J. Biol. Chem. 286 (35), 30481-30491 (2011).

NCBI and Uniprot Product Information

• NCBI GI #: 241110
• Molecular Weight: >110
• NCBI Official Full Name: dystrophin, partial
• NCBI Official Synonym Full Names: dystrophin
• NCBI Official Symbol: DMD
• NCBI Official Synonym Symbols: BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
• NCBI Protein Information: dystrophin
• UniProt Protein Name: Dystrophin
• Protein Family: Dystrophin
• UniProt Gene Name: DMD

NCBI Description

This gene spans a genomic range of greater than 2 Mb and encodes a large protein containing an N-terminal actin-binding domain and multiple spectrin repeats. The encoded protein forms a component of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton and the extracellular matrix. Deletions, duplications, and point mutations at this gene locus may cause Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), or cardiomyopathy. Alternative promoter usage and alternative splicing result in numerous distinct transcript variants and protein isoforms for this gene. [provided by RefSeq, Dec 2016]

Uniprot Description

dystrophin: Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin- associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission. Defects in DMD are the cause of Duchenne muscular dystrophy (DMD). DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. Defects in DMD are the cause of Becker muscular dystrophy (BMD). BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. Defects in DMD are a cause of cardiomyopathy dilated X- linked type 3B (CMD3B); also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: Cytoskeletal; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: Xp21.2-p21.1

Cellular Component: cell surface; cell-matrix junction; costamere; cytosol; dystrophin-associated glycoprotein complex; filopodium; filopodium membrane; lateral plasma membrane; lipid raft; protein complex; sarcolemma; synapse; syntrophin complex; Z disc

Molecular Function: actin binding; myosin binding; nitric-oxide synthase binding; protein binding; structural constituent of cytoskeleton; structural constituent of muscle; vinculin binding

Biological Process: cardiac muscle contraction; cellular protein complex assembly; muscle fiber development; muscle filament sliding; muscle maintenance; negative regulation of peptidyl-serine phosphorylation; peptide biosynthetic process; regulation of heart rate; regulation of skeletal muscle contraction; regulation of skeletal muscle contraction via regulation of the release of sequestered calcium ion

Disease: Cardiomyopathy, Dilated, 3b; Muscular Dystrophy, Becker Type; Muscular Dystrophy, Duchenne Type