人 FUCA1 抗体对
01AB00822
Alpha-L-fucosidase 1 (FUCA1) is an enzyme encoded by the FUCA1 gene, playing a crucial role in the hydrolysis of fucose residues from glycoproteins and glycolipids. It belongs to the glycoside hydrolase family and adopts a (β/α)8-barrel fold typical of GH29 enzymes, with its active site located at the core of the barrel. FUCA1's activity is essential for maintaining proper glycan degradation and remodeling, which are vital for cellular communication and immune responses. Structural studies have revealed its specificity for α(1,2)-, α(1,3)-, α(1,4)-, and α(1,6)-fucosylated linkages, highlighting its modular organization and catalytic efficiency. Mutations or deficiencies in FUCA1 lead to fucosidosis, a rare lysosomal storage disorder characterized by progressive neurological degeneration, growth retardation, and skeletal abnormalities due to impaired fucose metabolism. Reduced FUCA1 expression has also been implicated in colorectal tumors.
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技术参数
- 靶点基因名
- FUCA1
- 蛋白别名
- alpha-L-fucosidase 1; FUCA1
- UniProt
- P04066
- 物种
- Human
产品详情
- 靶点名称
- FUCA1
- 蛋白名称
- alpha-L-fucosidase 1
- 蛋白别名
- Tissue alpha-L-fucosidase; Alpha-L-fucosidase I; Alpha-L-fucoside fucohydrolase 1; Alpha-L-fucosidase 1
- 靶点基因名
- FUCA1
- UniProt
- P04066
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Alpha-L-fucosidase 1 (FUCA1) is an enzyme encoded by the FUCA1 gene, playing a crucial role in the hydrolysis of fucose residues from glycoproteins and glycolipids. It belongs to the glycoside hydrolase family and adopts a (β/α)8-barrel fold typical of GH29 enzymes, with its active site located at the core of the barrel. FUCA1's activity is essential for maintaining proper glycan degradation and remodeling, which are vital for cellular communication and immune responses. Structural studies have revealed its specificity for α(1,2)-, α(1,3)-, α(1,4)-, and α(1,6)-fucosylated linkages, highlighting its modular organization and catalytic efficiency. Mutations or deficiencies in FUCA1 lead to fucosidosis, a rare lysosomal storage disorder characterized by progressive neurological degeneration, growth retardation, and skeletal abnormalities due to impaired fucose metabolism. Reduced FUCA1 expression has also been implicated in colorectal tumors.