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DMD

DMD,即杜氏肌營養(yǎng)不良癥,是一種X染色體隱性遺傳疾病,由DMD基因突變引起,該基因位于X染色體的Xp21.1-3區(qū),編碼抗肌萎縮蛋白dystrophin。DMD的作用機制主要是由于dystrophin蛋白的缺乏或功能異常,導(dǎo)致肌細胞膜的穩(wěn)定性受損,進而引發(fā)肌肉纖維的壞死和炎癥反應(yīng)。在信號傳導(dǎo)方面,DMD的發(fā)病過程涉及到多種信號通路,包括肌肉細胞內(nèi)的鈣離子調(diào)節(jié)失衡、炎癥細胞因子的激活以及纖維化過程相關(guān)的TGF-β信號通路。在生物學(xué)意義上,DMD對肌肉組織的維持和功能至關(guān)重要,其異常通常導(dǎo)致進行性肌肉退化和無力,嚴重影響患者的運動能力和生活質(zhì)量。

熱銷產(chǎn)品

Recombinant Rat Dystrophin (Dmd), partial (CSB-YP006963RA)

驗證數(shù)據(jù)

CSB-YP006963RA

(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

DMD Recombinant Monoclonal Antibody (CSB-RA271553A0HU)

驗證數(shù)據(jù)

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)