人 NAGA 抗体对
01AB01244
Alpha-N-acetylgalactosaminidase (NAGA), also known as nagalase, is a lysosomal exoglycosidase enzyme that specifically removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides, playing a crucial role in the degradation of glycolipids and glycoproteins within the lysosome. Structurally, NAGA shares conserved active site residues with the closely related alpha-galactosidase A, and its catalytic mechanism has been elucidated through X-ray crystallography, revealing the basis for substrate specificity and the effects of pathogenic mutations. NAGA is also notable for its potential application in blood group conversion, as it can help produce type O "universal donor" blood from type A blood by removing specific carbohydrate residues. Deficiency or mutations in the NAGA gene lead to Schindler disease, a lysosomal storage disorder characterized by the accumulation of undegraded carbohydrates, resulting in neurological and systemic symptoms.
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技术参数
- 靶点基因名
- NAGA
- 蛋白别名
- alpha-N-acetylgalactosaminidase; NAGA
- UniProt
- P17050
- 物种
- Human
产品详情
- 靶点名称
- NAGA
- 蛋白名称
- alpha-N-acetylgalactosaminidase
- 蛋白别名
- Alpha-N-acetylgalactosaminidase; Alpha-galactosidase B
- 靶点基因名
- NAGA
- UniProt
- P17050
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Alpha-N-acetylgalactosaminidase (NAGA), also known as nagalase, is a lysosomal exoglycosidase enzyme that specifically removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides, playing a crucial role in the degradation of glycolipids and glycoproteins within the lysosome. Structurally, NAGA shares conserved active site residues with the closely related alpha-galactosidase A, and its catalytic mechanism has been elucidated through X-ray crystallography, revealing the basis for substrate specificity and the effects of pathogenic mutations. NAGA is also notable for its potential application in blood group conversion, as it can help produce type O "universal donor" blood from type A blood by removing specific carbohydrate residues. Deficiency or mutations in the NAGA gene lead to Schindler disease, a lysosomal storage disorder characterized by the accumulation of undegraded carbohydrates, resulting in neurological and systemic symptoms.