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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

CSB-YP006963RA

(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

CSB-RA271553A0HU

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.

CSB-RA271553A0HU

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)

dmd Proteins

dmd Proteins for Mus musculus (Mouse)

dmd Proteins for Gallus gallus (Chicken)

dmd Proteins for Rattus norvegicus (Rat)

dmd Proteins for Homo sapiens (Human)

dmd Proteins for Canis familiaris (Dog) (Canis lupus familiaris)

dmd Proteins for Enterobacteria phage T4 (Bacteriophage T4)

dmd Proteins for Sus scrofa (Pig)

dmd Proteins for Hyphomicrobium sp. (strain x)