Reactivity: | Human, Mouse, Rat |
Applications: | WB, IHC, IF/IC, ELISA |
Host Species: | Rabbit |
Isotype: | IgG |
Clonality: | Polyclonal antibody |
Gene Name: | dystrophin |
Gene Symbol: | DMD |
Synonyms: | BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Dystrophin |
Gene ID: | 1756 |
UniProt ID: | P11532 |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 346-635 of human Dystrophin (NP_003997.2). |
Dilution: | WB 1:500-1:1000; IHC 1:50-1:200; IF/IC 1:50-1:200 |
Purification Method: | Affinity purification |
Concentration: | 0.46 mg/ml |
Buffer: | PBS with 0.05% proclin300, 50% glycerol, pH7.3. |
Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
Documents: | Manual-DMD antibody |
Background
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.
Images
![]() | Western blot analysis of various lysates using Dystrophin Rabbit pAb (A1411) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 10s. |
![]() | Immunohistochemistry analysis of paraffin-embedded Human skeletal muscle using Dystrophin Rabbit pAb (A1411) at dilution of 1:20 (40x lens). High pressure antigen retrieval performed with 0.01M Citrate Bufferr (pH 6.0) prior to IHC staining. |
![]() | Immunohistochemistry analysis of paraffin-embedded Mouse heart using Dystrophin Rabbit pAb (A1411) at dilution of 1:20 (40x lens). High pressure antigen retrieval performed with 0.01M Citrate Bufferr (pH 6.0) prior to IHC staining. |
![]() | Immunohistochemistry analysis of paraffin-embedded Rat heart using Dystrophin Rabbit pAb (A1411) at dilution of 1:20 (40x lens). High pressure antigen retrieval performed with 0.01M Citrate Bufferr (pH 6.0) prior to IHC staining. |
![]() | Immunofluorescence analysis of paraffin-embedded Human skeletal muscle using Dystrophin Rabbit pAb (A1411) at dilution of 1:50 (40x lens). Secondary antibody: Cy3-conjugated Goat anti-Rabbit IgG (H+L) (AS007) at 1:500 dilution. Blue: DAPI for nuclear staining.Perform high pressure antigen retrieval with 0.01 M citrate buffer (pH 6.0) prior to IF staining. |
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