FUCA1,α-L岩藻糖苷酶抗体

产品编号HZ-2940R
英文名称FUCA1
中文名称α-L岩藻糖苷酶抗体
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-fucosidase.说 明 书0.1ml 0.2ml
研究领域肿瘤 细胞生物 免疫学 信号转导 肿瘤细胞生物标志物
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse,
FUCA1,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.
分 子 量50kDa
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human FUCA1/Alpha L fucosidase I
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
FUCA1,α-L岩藻糖苷酶抗体PubMedPubMed
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.

FUCA1,α-L岩藻糖苷酶抗体DISEASE:
Fucosidosis (FUCA1D) [MIM:230000]: An autosomal recessive lysosomal storage 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 links:
UniProtKB/Swiss-Prot: P04066.4

FUCA1,α-L岩藻糖苷酶抗体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。