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Rabbit Anti-FUCA1/RBITC Conjugated antibody (bs-2940R-RBITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-2940R-RBITC
英文名稱 Rabbit Anti-FUCA1/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的α-L巖藻糖苷酶抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  腫瘤細(xì)胞生物標(biāo)志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 50kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FUCA1/Alpha L fucosidase I
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 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 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:

Entrez Gene: 2517 Human

Entrez Gene: 71665 Mouse

Entrez Gene: 24375 Rat

Omim: 612280 Human

SwissProt: P04066 Human

SwissProt: Q99LJ1 Mouse

SwissProt: P17164 Rat

Unigene: 370858 Human

Unigene: 439940 Mouse

Unigene: 3469 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

α-L-巖藻糖苷酶是一種催化含巖藻糖基的糖蛋白、糖脂等生物活性大分子水解酶的溶酶體酸性水解酶。其廣泛分布于人體組織細(xì)胞、血液和體液中。參與體內(nèi)糖蛋白、糖脂和寡糖的代謝。由于肝癌患者α-L-巖藻糖苷酶明顯升高,目前它被認(rèn)為是原發(fā)性肝癌的一種新的腫瘤標(biāo)記物。α-L巖藻糖苷酶升高:見于原發(fā)性肝癌、轉(zhuǎn)移性肝癌、肝硬化、急性肝炎等。
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