DMD, or Duchenne muscular dystrophy, is an X-chromosome recessive disorder caused by mutations in the DMD gene, which is located in the Xp21.1-3 region of the X chromosome and encodes the antimyotrophic protein dystrophin.The mechanism of action of DMD is primarily due to the absence or abnormal function of the dystrophin protein, which results in impaired stability of the myocyte membrane, which in turn triggers necrosis and inflammatory response of muscle fibres. In terms of signalling, the pathogenesis of DMD involves a variety of signalling pathways, including an imbalance in calcium regulation within the muscle cell, activation of inflammatory cytokines, and the TGF-β signalling pathway associated with the fibrotic process. Biologically, DMD is critical for the maintenance and function of muscle tissue, and its abnormalities usually lead to progressive muscle degeneration and weakness, which severely affects the patient's exercise capacity and quality of life.
Recombinant Rat Dystrophin (Dmd), partial (CSB-YP006963RA)
Validated Data
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
DMD Recombinant Monoclonal Antibody (CSB-RA271553A0HU)
Validated Data
IHC image of CSB-RA271553A0HU diluted at 1:100 and staining in paraffin-embedded human heart tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
IHC image of CSB-RA271553A0HU diluted at 1:100 and staining in paraffin-embedded human skeletal muscle tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
The following Dmd reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
Dmd Antibodies for Homo sapiens (Human)
Code | Product Name | Species Reactivity | Application |
---|---|---|---|
CSB-PA006963GA01HU | DMD Antibody |
Human,Mouse,Rat | ELISA,WB |
CSB-PA503789 | DMD Antibody |
Human,Mouse | ELISA,IHC |
CSB-PA006963LA01HU | DMD Antibody |
Human | ELISA, IHC |
CSB-PA006963LB01HU | DMD Antibody, HRP conjugated |
Human | ELISA |
CSB-PA006963LD01HU | DMD Antibody, Biotin conjugated |
Human | ELISA |
CSB-RA271553A0HU | DMD Recombinant Monoclonal Antibody |
Human | ELISA, IHC |
Dmd Antibodies for Enterobacteria phage T4 (Bacteriophage T4)
Code | Product Name | Species Reactivity | Application |
---|---|---|---|
CSB-PA330993XA01EDZ | dmd Antibody |
Enterobacteria phage T4 (Bacteriophage T4) | ELISA, WB (ensure identification of antigen) |
Dmd Proteins for Mus musculus (Mouse)
Code | Product Name | Source |
---|---|---|
CSB-YP006963MO CSB-EP006963MO CSB-BP006963MO CSB-MP006963MO CSB-EP006963MO-B |
Recombinant Mouse Dystrophin (Dmd), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
Dmd Proteins for Gallus gallus (Chicken)
Code | Product Name | Source |
---|---|---|
CSB-YP006963CH CSB-EP006963CH CSB-BP006963CH CSB-MP006963CH CSB-EP006963CH-B |
Recombinant Chicken Dystrophin (DMD), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
Dmd Proteins for Rattus norvegicus (Rat)
Code | Product Name | Source |
---|---|---|
CSB-BP006963RA CSB-MP006963RA CSB-EP006963RA-B |
Recombinant Rat Dystrophin (Dmd), partial |
Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
Dmd Proteins for Homo sapiens (Human)
Code | Product Name | Source |
---|---|---|
CSB-YP006963HU CSB-EP006963HU CSB-BP006963HU CSB-MP006963HU CSB-EP006963HU-B |
Recombinant Human Dystrophin (DMD), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
Dmd Proteins for Canis lupus familiaris (Dog) (Canis familiaris)
Code | Product Name | Source |
---|---|---|
CSB-YP006963DO CSB-EP006963DO CSB-BP006963DO CSB-MP006963DO CSB-EP006963DO-B |
Recombinant Dog Dystrophin (DMD), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
Dmd Proteins for Enterobacteria phage T4 (Bacteriophage T4)
Code | Product Name | Source |
---|---|---|
CSB-YP330993EDZ CSB-EP330993EDZ CSB-BP330993EDZ CSB-MP330993EDZ CSB-EP330993EDZ-B |
Recombinant Enterobacteria phage T4 Uncharacterized 7.0 kDa protein in sp-Gp41 intergenic region (y02B) |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
Dmd Proteins for Sus scrofa (Pig)
Code | Product Name | Source |
---|---|---|
CSB-YP006963PI CSB-EP006963PI CSB-BP006963PI CSB-MP006963PI CSB-EP006963PI-B |
Recombinant Pig Dystrophin (DMD), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
Dmd Proteins for Hyphomicrobium sp. (strain x)
Code | Product Name | Source |
---|---|---|
CSB-YP675252HBAL CSB-EP675252HBAL CSB-BP675252HBAL CSB-MP675252HBAL CSB-EP675252HBAL-B |
Recombinant Hyphomicrobium sp. Dimethylamine dehydrogenase (dmd), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |