α-L岩藻糖苷酶抗体
规格：1mg/1ml
英文名： FUCA1
别名： Alpha L fucosidase I; Alpha L fucoside fucohydrolase; Alpha-L-fucosidase 1; Alpha-L-fucosidase I; Alpha-L-fucoside fucohydrolase 1; FUCA; FUCA1; FUCO_HUMAN; Tissue alpha L fucosidase; Tissue alpha-L-f
分子量： 50kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human FUCA1/Al
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse,
细胞定位：
α-L岩藻糖苷酶抗体产品介绍：background: Alpha-L-fucosidase,AFU is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. Function: Alpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. Subunit: Homotetramer. Subcellular Location: Lysosome. DISEASE: Fucosidosis (FUCA1D) [MIM:230000]: An autosomal recessive lysosomal storageα-L岩藻糖苷酶抗体 disease characterized by accumulation of fucose-containing glycolipids and glycoproteins in various tissues. Clinical signs include facial dysmorphism, dysostosis multiplex, moderate hepatomegaly, severe intellectual deficit, deafness, and according to age, angiokeratomas. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the glycosyl hydrolase 29 family. Database α-L岩藻糖苷酶抗体links: UniProtKB/Swiss-Prot: P04066.4 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. α-L-岩藻糖苷酶是一种催化含岩藻糖基的糖蛋白、糖脂等生物活性大分子水解酶的溶酶体酸性水解酶。其广泛分布于人体组织细胞、血液和体液中。参与体内糖蛋白、糖脂和寡糖的代谢。由于肝癌患者α-L-岩藻糖苷酶明显升高，目前它被认为是原发性肝癌的一种新的肿瘤标记物。α-L岩藻糖苷酶升高：见于原发性肝癌、转移性肝癌、肝硬化、急性肝炎等。
α-L岩藻糖苷酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导 肿瘤细胞生物标志物
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid