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Rabbit Anti-ITGB4/CD104/BF350 Conjugated antibody (bs-10028R-BF350)
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-10028R-BF350
英文名稱 Rabbit Anti-ITGB4/CD104/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的整合素β4抗體
別    名 Integrin beta 4; Integrin beta4; ITGB4; ITGB-4; CD 104; CD104; CD104 antigen; GP150; Integrin beta-4; ITB4_HUMAN; ITG B4; ITGB 4; ITGB4.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞凋亡  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse,  (predicted: Rat, Cow, Rabbit, Guinea Pig, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 197kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ITGB4
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:
Integrins are heterodimers comprised of alpha and beta subunits, that are noncovalently associated transmembrane glycoprotein receptors. Different combinations of alpha and beta polypeptides form complexes that vary in their ligand-binding specificities. Integrins mediate cell-matrix or cell-cell adhesion, and transduced signals that regulate gene expression and cell growth. This gene encodes the integrin beta 4 subunit, a receptor for the laminins. This subunit tends to associate with alpha 6 subunit and is likely to play a pivotal role in the biology of invasive carcinoma. Mutations in this gene are associated with epidermolysis bullosa with pyloric atresia. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Function:
Integrin alpha-6/beta-4 is a receptor for laminin. It plays a critical structural role in the hemidesmosome of epithelial cells. Is required for the regulation of keratinocyte polarity and motility.

Subunit:
Heterodimer of an alpha and a beta subunit. Beta-4 associates with alpha-6. Interacts (via cytoplasmic region) with COL17A1 (via cytoplasmic region). Interacts (via cytoplasmic region) with DST isoform 3 (via N-terminus). Interacts (via cytoplasmic domain) with DST (via N-terminus). Interacts with RAC1.

Subcellular Location:
Membrane; Single-pass type I membrane protein. Cell junction, hemidesmosome. Note=Colocalizes with DST at the leading edge of migrating keratinocytes.

Tissue Specificity:
Integrin alpha-6/beta-4 is predominantly expressed by epithelia. Isoform beta-4D is also expressed in colon and placenta. Isoform beta-4E is also expressed in epidermis, lung, duodenum, heart, spleen and stomach.

DISEASE:
Defects in ITGB4 are a cause of epidermolysis bullosa letalis with pyloric atresia (EB-PA) [MIM:226730]; also known as junctional epidermolysis bullosa with pyloric atresia (PA-JEB) or aplasia cutis congenita with gastrointestinal atresia. EB-PA is an autosomal recessive, frequently lethal, epidermolysis bullosa with variable involvement of skin, nails, mucosa, and with variable effects on the digestive system. It is characterized by mucocutaneous fragility, aplasia cutis congenita, and gastrointestinal atresia, which most commonly affects the pylorus. Pyloric atresia is a primary manifestation rather than a scarring process secondary to epidermolysis bullosa.
Defects in ITGB4 are a cause of generalized atrophic benign epidermolysis bullosa (GABEB) [MIM:226650]. GABEB is a non-lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities.

Similarity:
Belongs to the integrin beta chain family.
Contains 1 Calx-beta domain.
Contains 4 fibronectin type-III domains.
Contains 1 PSI domain.
Contains 1 VWFA domain.

Database links:

Entrez Gene: 3691 Human

Entrez Gene: 192897 Mouse

Entrez Gene: 25724 Rat

Omim: 147557 Human

SwissProt: P16144 Human

SwissProt: A2A863 Mouse

SwissProt: Q64632 Rat

Unigene: 632226 Human

Unigene: 213873 Mouse

Unigene: 198908 Rat



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