DMD
DMD，即杜氏肌營養不良癥，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，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，導致肌細胞膜的穩定性受損，進而引發肌肉纖維的壞死和炎癥反應。在信號傳導方面，DMD的發病過程涉及到多種信號通路，包括肌肉細胞內的鈣離子調節失衡、炎癥細胞因子的激活以及纖維化過程相關的TGF-β信號通路。在生物學意義上，DMD對肌肉組織的維持和功能至關重要，其異常通常導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量。
熱銷產品
Recombinant Rat Dystrophin (Dmd), partial (CSB-YP006963RA)
驗證數據
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
DMD Recombinant Monoclonal Antibody (CSB-RA271553A0HU)
驗證數據
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)
| 產品貨號 | 產品名稱 | 種屬反應性 | 應用類型 |
|---|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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 |
