人 AGBL1 抗体对
01AB02582
AGBL carboxypeptidase 1 (AGBL1), also known as cytosolic carboxypeptidase 4 (CCP4), is a metallocarboxypeptidase that catalyzes the deglutamylation of polyglutamylated proteins, primarily tubulin. It removes polyglutamate side chains from the C-terminal tail of tubulin, thereby modulating microtubule post-translational modifications and stability. This activity is essential for regulating microtubule-based cellular processes and protein metabolism. Clinically, pathogenic variants in AGBL1 are associated with autosomal dominant late-onset Fuchs endothelial corneal dystrophy (FECD8). This degenerative condition is characterized by progressive loss of corneal endothelial cells, thickening of the Descemet membrane, and corneal edema, eventually leading to significant visual impairment.
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技术参数
- 靶点基因名
- AGBL1
- 基因别名
- CCP4
- 蛋白别名
- AGBL carboxypeptidase 1; AGBL1
- UniProt
- Q96MI9
- 物种
- Human
产品详情
- 靶点名称
- AGBL1
- 蛋白名称
- AGBL carboxypeptidase 1
- 蛋白别名
- Cytosolic carboxypeptidase 4; ATP/GTP-binding protein-like 1; Protein deglutamylase CCP4
- 靶点基因名
- AGBL1
- 基因别名
- CCP4
- UniProt
- Q96MI9
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
AGBL carboxypeptidase 1 (AGBL1), also known as cytosolic carboxypeptidase 4 (CCP4), is a metallocarboxypeptidase that catalyzes the deglutamylation of polyglutamylated proteins, primarily tubulin. It removes polyglutamate side chains from the C-terminal tail of tubulin, thereby modulating microtubule post-translational modifications and stability. This activity is essential for regulating microtubule-based cellular processes and protein metabolism. Clinically, pathogenic variants in AGBL1 are associated with autosomal dominant late-onset Fuchs endothelial corneal dystrophy (FECD8). This degenerative condition is characterized by progressive loss of corneal endothelial cells, thickening of the Descemet membrane, and corneal edema, eventually leading to significant visual impairment.