人 SERPINB6 抗体对
01AB02100
Serpin family B member 6 (SERPINB6) is a cytosolic, non-secreted serine protease inhibitor belonging to the clade B (ovalbumin-like) serpin subfamily. It functions by inhibiting various proteases, including cathepsin G, kallikrein-8, thrombin, and β-tryptase, thereby protecting cells from proteolytic damage during inflammation and cellular stress. SERPINB6 is broadly expressed, with significant levels found in inner ear hair cells, the brain, and mast cells, where it regulates endogenous protease activity. Structurally, it utilizes a reactive center loop as a suicide substrate to neutralize target enzymes. Clinically, biallelic loss-of-function mutations in SERPINB6 cause autosomal recessive nonsyndromic sensorineural deafness (DFNB91). This condition results from the increased vulnerability of inner ear hair cells to lysosomal leakage and protease-mediated cell death, highlighting the protein's essential role in maintaining auditory function.
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技术参数
- 靶点基因名
- SERPINB6
- 基因别名
- PI6; PTI
- 蛋白别名
- serpin family B member 6; SERPINB6
- UniProt
- P35237
- 物种
- Human
产品详情
- 靶点名称
- SERPINB6
- 蛋白名称
- serpin family B member 6
- 蛋白别名
- Serpin B6; Cytoplasmic antiproteinase; CAP; Peptidase inhibitor 6; PI-6; Placental thrombin inhibitor
- 靶点基因名
- SERPINB6
- 基因别名
- PI6; PTI
- UniProt
- P35237
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Serpin family B member 6 (SERPINB6) is a cytosolic, non-secreted serine protease inhibitor belonging to the clade B (ovalbumin-like) serpin subfamily. It functions by inhibiting various proteases, including cathepsin G, kallikrein-8, thrombin, and β-tryptase, thereby protecting cells from proteolytic damage during inflammation and cellular stress. SERPINB6 is broadly expressed, with significant levels found in inner ear hair cells, the brain, and mast cells, where it regulates endogenous protease activity. Structurally, it utilizes a reactive center loop as a suicide substrate to neutralize target enzymes. Clinically, biallelic loss-of-function mutations in SERPINB6 cause autosomal recessive nonsyndromic sensorineural deafness (DFNB91). This condition results from the increased vulnerability of inner ear hair cells to lysosomal leakage and protease-mediated cell death, highlighting the protein's essential role in maintaining auditory function.