抗人 DNAH5 抗体对
01AB01957
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.
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技术参数
- 靶点基因名
- DNAH5
- 基因别名
- DNAHC5; HL1; KIAA1603
- 蛋白别名
- dynein axonemal heavy chain 5; DNAH5
- UniProt
- Q8TE73
- 物种
- Human
产品详情
- 靶点名称
- DNAH5
- 蛋白名称
- dynein axonemal heavy chain 5
- 蛋白别名
- Dynein axonemal heavy chain 5; Axonemal beta dynein heavy chain 5; Ciliary dynein heavy chain 5
- 靶点基因名
- DNAH5
- 基因别名
- DNAHC5; HL1; KIAA1603
- UniProt
- Q8TE73
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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.