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Rabbit Anti-TNXB/RBITC Conjugated antibody (bs-18163R-RBITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-18163R-RBITC
英文名稱 Rabbit Anti-TNXB/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的腱糖蛋白X抗體
別    名 Ehlers Danlos like syndrome; Hexabrachion like protein; Hexabrachion-like protein; HXBL; NXB2; Tenascin X precursor; Tenascin XB; Tenascin XB1; Tenascin XB2; Tenascin-X; TENX; TENX_HUMAN; TN X; TN-X; TNX; TNXB; TNXB1; TNXB2; TNXBS; XB; XBS.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  信號轉導  細胞粘附分子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
產品應用 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 462kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TNXB
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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.
產品介紹 background:
This gene encodes a member of the tenascin family of extracellular matrix glycoproteins. The tenascins have anti-adhesive effects, as opposed to fibronectin which is adhesive. This protein is thought to function in matrix maturation during wound healing, and its deficiency has been associated with the connective tissue disorder Ehlers-Danlos syndrome. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. It is one of four genes in this cluster which have been duplicated. The duplicated copy of this gene is incomplete and is a pseudogene which is transcribed but does not encode a protein. The structure of this gene is unusual in that it overlaps the CREBL1 and CYP21A2 genes at its 5' and 3' ends, respectively. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Function:
Appears to mediate interactions between cells and the extracellular matrix. Substrate-adhesion molecule that appears to inhibit cell migration. Accelerates collagen fibril formation. May play a role in supporting the growth of epithelial tumors.

Subcellular Location:
Secreted, extracellular space, extracellular matrix.

Tissue Specificity:
Highly expressed in fetal adrenal, in fetal testis, fetal smooth, striated and cardiac muscle. Isoform XB-short is only expressed in the adrenal gland.

DISEASE:
Tenascin-X deficiency (TNXD) [MIM:606408]: TNXD leads to an Ehlers-Danlos-like syndrome characterized by hyperextensible skin, hypermobile joints, and tissue fragility. Tenascin-X-deficient patients, however, lack atrophic scars, a major diagnostic criteria for classic Ehlers-Danlos. Delayed wound healing, which is also common in classic EDS, is only present in a subset of patients. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the tenascin family.
Contains 19 EGF-like domains.
Contains 1 fibrinogen C-terminal domain.
Contains 32 fibronectin type-III domains.

Database links:

Entrez Gene: 7148 Human

Entrez Gene: 81877 Mouse

Omim: 600985 Human

SwissProt: P22105 Human

Unigene: 485104 Human

Unigene: 290527 Mouse



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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