徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
DNAH5 Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611846 |
| 100ul | WGD-Z-2611846 |
详细参数
| 别名 | Dynein axonemal heavy chain 5; Axonemal beta dynein heavy chain 5; Ciliary dynein heavy chain 5 |
| 基因名 | DNAH5 |
| UniProt ID | Q8TE73 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1J1-Y3-A4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 529 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:500-1:1000 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Dynein axonemal heavy chain 5 (DNAH5) is a large ATP-dependent microtubule motor protein that serves as a primary component of the outer dynein arm in motile cilia. It generates the minus-end–directed force required for ciliary beating and fluid transport, which is essential for the clearance of mucus in the respiratory tract and the movement of ependymal cells. DNAH5 is critical for the structural and functional integrity of the axoneme. Pathogenic variants in the DNAH5 gene are a leading cause of autosomal recessive primary ciliary dyskinesia type 3. This condition is characterized by chronic sinopulmonary infections, bronchiectasis, and laterality defects such as situs inversus, often manifesting as Kartagener syndrome due to the loss or shortening of outer dynein arms. |