Dmd
DMD,即杜氏肌营养不良症,是一种X染色体隐性遗传疾病,由DMD基因突变引起,该基因位于X染色体的Xp21.1-3区,编码抗肌萎缩蛋白dystrophin。DMD的作用机制主要是由于dystrophin蛋白的缺乏或功能异常,导致肌细胞膜的稳定性受损,进而引发肌肉纤维的坏死和炎症反应。在信号传导方面,DMD的发病过程涉及到多种信号通路,包括肌肉细胞内的钙离子调节失衡、炎症细胞因子的激活以及纤维化过程相关的TGF-β信号通路。在生物学意义上,DMD对肌肉组织的维持和功能至关重要,其异常通常导致进行性肌肉退化和无力,严重影响患者的运动能力和生活质量。
热销产品
Recombinant Rat Dystrophin (Dmd), partial (CSB-YP006963RA)
验证数据
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
DMD Recombinant Monoclonal Antibody (CSB-RA271553A0HU)
验证数据
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.
Dmd Antibodies
Dmd for Homo sapiens (Human)
产品货号 | 产品名称 | 种属反应性 | 应用类型 |
---|---|---|---|
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 Proteins
Dmd Proteins for Mus musculus (Mouse)
产品货号 | 产品名称 | 来源 |
---|---|---|
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)
产品货号 | 产品名称 | 来源 |
---|---|---|
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)
产品货号 | 产品名称 | 来源 |
---|---|---|
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)
产品货号 | 产品名称 | 来源 |
---|---|---|
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 familiaris (Dog) (Canis lupus familiaris)
产品货号 | 产品名称 | 来源 |
---|---|---|
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)
产品货号 | 产品名称 | 来源 |
---|---|---|
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)
产品货号 | 产品名称 | 来源 |
---|---|---|
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)
产品货号 | 产品名称 | 来源 |
---|---|---|
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 |