腱糖蛋白X抗体
规格：1mg/1ml
英文名： TNXB
别名： 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;
分子量： 462kDa
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 TNXB
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆 分泌型蛋白
腱糖蛋白X抗体产品介绍：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 腱糖蛋白X抗体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 腱糖蛋白X抗体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. Gene ID: 7148 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.
腱糖蛋白X抗体产品应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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：细胞生物 信号转导 细胞粘附分子
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid