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anti-DMD antibody :: Rabbit Dystrophin Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS301209
Unit / Price
0.1 mL (Concentrate)  /  $165 +1 FREE 8GB USB
7 mL (RTU)  /  $225 +1 FREE 8GB USB
0.5 mL (Concentrate)  /  $335 +1 FREE 8GB USB
1 mL (Concentrate)  /  $425 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Dystrophin (DMD), Polyclonal Antibody

 Also Known As   

Rabbit Anti-Human Dystrophin Polyclonal Antibody

 Product Gene Name   

anti-DMD antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    300376
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse
Predicted Reactivity: Bovine, Chicken, Dog, Pig
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 Form/Format    0.1 ml immunogen affinity purified rabbit polyclonal antibody in PBS/1% BSA buffer pH 7.6 with less than 0.1% sodium azide.
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 Immunogen    Synthetic peptide derived from C-terminus of human dystrophin.
 Control Tissue    Skeletal Muscle
 Cellular Localization    Membrane
 Entrez Gene Code    1756
 Pathway    Muscle Markers
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 Preparation and Storage    Store at 2-8 degree C. Do not freeze. The user must validate any other storage conditions. When properly stored, the reagent is stable to the date indicated on the label. Do not use the reagent beyond the expiration date. There are no definitive signs to indicate instability of this product; therefore, positive and negative controls should be tested simultaneously with unknown specimens. If unexpected results are observed which cannot be explained by variations in laboratory procedures and a problem with the reagent is suspected, contact Technical Support.
 Other Notes    Small volumes of anti-DMD antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for anti-DMD antibody

   Dystrophin is the 427kDa protein product of the DMB/BMD gene located on the X chromosome at position Xp21. Western blotting and immunohistochemistry are the two established methods for the detection of abnormalities of dystrophin expression in muscle biopsies. Dystrophin abnormalities are thought to occur in 100% of patients with DMD/BMD, although genetic abnormalities may only be detected in up to 65% of cases.
 Applications Tested/Suitable for anti-DMD antibody   

Immunohistochemistry (IHC) Paraffin, Western Blot (WB)

 Application Notes for anti-DMD antibody    Immunohistochemistry Procedure
Specimen Preparation: Formalin-fixed, paraffin-embedded tissues are suitable for use with this primary antibody. Deparaffinization: Deparaffinize slides using xylene or xylene alternative and graded alcohols. Antibody Dilution: If using the concentrate format of this product, dilute the antibody 1:100. The dilutions are estimates; actual results may differ because of variability in methods and protocols. Antigen Retrieval: Boil tissue section in 10mM Citrate buffer, pH 6.0 for 10 min followed by cooling at room temperature for 20 min. Primary Antibody Incubation: Incubate for 10 minutes at room temperature. Slide Washing: Slides must be washed in between steps. Rinse slides with PBS/0.05% Tween. Visualization: Detect the antibody as instructed by the instructions provided with the visualization system.
Immunohistochemistry Dilution: 1:100
IHC Positive Control: Skeletal Muscle

Western Blot
Recommended Starting Protocol: Dilute the antibody 1:25. Incubate for 1 hour at room temperature. The dilution is an estimate; actual results may differ because of variability in methods and protocols. Optimal dilution and procedure should be determined by the end user.
Western Blot Positive Control: Skeletal muscle
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 Western Blot (WB) of anti-DMD antibody    Western Blot analysis of skeletal muscle skeletal muscle antibody
anti-DMD antibody Western Blot (WB) (WB) image
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 Immunohistochemistry (IHC) of anti-DMD antibody    Human skeletal muscle stained with anti-dystrophin antibody
anti-DMD antibody Immunohistochemistry (IHC) (IHC) image
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NCBI/Uniprot data below describe general gene information for DMD. It may not necessarily be applicable to this product.
 NCBI GI #    5032281
 NCBI GeneID    1756
 NCBI Accession #    NP_000100.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000109.3 [Other Products]
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 UniProt Secondary Accession #    Q02295; Q14169; Q14170; Q5JYU0; Q6NSJ9; Q7KZ48; Q8N754; Q9UCW3; Q9UCW4; E9PDN1 [Other Products]
 UniProt Related Accession #    P11532 [Other Products]
 Molecular Weight    427 kDa [Similar Products]
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 NCBI Official Full Name    dystrophin Dp427c isoform
 NCBI Official Synonym Full Names    dystrophin
 NCBI Official Symbol    DMD [Similar Products]
 NCBI Official Synonym Symbols   
BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
[Similar Products]
 NCBI Protein Information    dystrophin
 UniProt Protein Name    Dystrophin
 Protein Family    Dystrophin
 UniProt Gene Name    DMD [Similar Products]
 UniProt Entry Name    DMD_HUMAN
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 NCBI Summary for DMD    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, Jul 2008]
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 UniProt Comments for DMD    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: Motility/polarity/chemotaxis; Cytoskeletal

Chromosomal Location of Human Ortholog: Xp21.2

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

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

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

Disease: Cardiomyopathy, Dilated, 3b; Muscular Dystrophy, Becker Type; Muscular Dystrophy, Duchenne Type
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 Research Articles on DMD    1. Novel mutations in the human dystrophin gene in Duchenne and Becker muscular dystrophy.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-DMD antibodyPathways associated with anti-DMD antibody
 Reference Product  PubMed Publications
 DAG1 antibody  >30 publications with DMD and DAG1
 TTN antibody  >11 publications with DMD and TTN
 CAV3 antibody  >7 publications with DMD and CAV3
 TNNT2 antibody  >4 publications with DMD and TNNT2
 SGCA antibody  >4 publications with DMD and SGCA
 TPM1 antibody  >2 publications with DMD and TPM1
 Products by Pathway  Pathway Diagram
 Arrhythmogenic Right Ventricular Cardiomyopathy Pathway antibodies  Arrhythmogenic Right Ventricular Cardiomyopathy Pathway Diagram
 Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway antibodies  Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway Diagram
 Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway antibodies  Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway Diagram
 Dilated Cardiomyopathy Pathway antibodies  Dilated Cardiomyopathy Pathway Diagram
 Dilated Cardiomyopathy Pathway antibodies  Dilated Cardiomyopathy Pathway Diagram
 Extracellular Matrix Organization Pathway antibodies  Extracellular Matrix Organization Pathway Diagram
 Hypertrophic Cardiomyopathy (HCM) Pathway antibodies  Hypertrophic Cardiomyopathy (HCM) Pathway Diagram
 Hypertrophic Cardiomyopathy (HCM) Pathway antibodies  Hypertrophic Cardiomyopathy (HCM) Pathway Diagram
 Muscle Contraction Pathway antibodies  Muscle Contraction Pathway Diagram
 Non-integrin Membrane-ECM Interactions Pathway antibodies  Non-integrin Membrane-ECM Interactions Pathway Diagram
Diseases associated with anti-DMD antibodyOrgans/Tissues associated with anti-DMD antibody
 Disease Name  Pubmed Publications
 Muscular Dystrophy, Duchenne Antibodies  >3760 publications with DMD and Muscular Dystrophy, Duchenne
 CARDIOMYOPATHY, DILATED, 3B Antibodies  >3760 publications with DMD and CARDIOMYOPATHY, DILATED, 3B
 Nervous System Diseases Antibodies  >3375 publications with DMD and Nervous System Diseases
 Muscular Dystrophies Antibodies  >3248 publications with DMD and Muscular Dystrophies
 Muscular Dystrophy, Animal Antibodies  >783 publications with DMD and Muscular Dystrophy, Animal
 Disease Models, Animal Antibodies  >359 publications with DMD and Disease Models, Animal
 Heart Diseases Antibodies  >288 publications with DMD and Heart Diseases
 Fibrosis Antibodies  >248 publications with DMD and Fibrosis
 Necrosis Antibodies  >211 publications with DMD and Necrosis
 Cardiomyopathies Antibodies  >158 publications with DMD and Cardiomyopathies
 Organ/Tissue Name  Pubmed Publications
 Muscle Antibodies  >2561 publications with DMD and Muscle
 Heart Antibodies  >436 publications with DMD and Heart
 Brain Antibodies  >309 publications with DMD and Brain
 Nerve Antibodies  >272 publications with DMD and Nerve
 Bone Antibodies  >146 publications with DMD and Bone
 Vascular Antibodies  >115 publications with DMD and Vascular
 Eye Antibodies  >106 publications with DMD and Eye
 Embryonic Tissue Antibodies  >102 publications with DMD and Embryonic Tissue
 Connective Tissue Antibodies  >94 publications with DMD and Connective Tissue
 Skin Antibodies  >84 publications with DMD and Skin
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