| 產(chǎn)品編號 | bs-17063R-BF594 |
| 英文名稱1 | Rabbit Anti-Kindlin/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的整合素相互作用蛋白Kindlin抗體 |
| 別 名 | C20orf42; Chromosome 20 open reading frame 42; DTGCU 2; DTGCU2; FERM1_HUMAN; Fermitin family homolog 1; Fermt1; FLJ20116; FLJ23423; KIND 1; KIND1; Kinderlin; Kindlerin; Kindlin 1; Kindlin syndrome protein; Kindlin-1; Kindlin1; Unc 112 related protein 1; Unc-112-related protein 1; Unc112 related protein; UNC112A; URP 1; URP1. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 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) | |
| 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 | 77kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Kindlin |
| 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(chǎn)品介紹 |
background: This gene encodes a member of the fermitin family, and contains a FERM domain and a pleckstrin homology domain. The encoded protein is involved in integrin signaling and linkage of the actin cytoskeleton to the extracellular matrix. Mutations in this gene have been linked to Kindler syndrome. [provided by RefSeq, Dec 2009] Function: Involved in cell adhesion. Contributes to integrin activation. When coexpressed with talin, potentiates activation of ITGA2B. Required for normal keratinocyte proliferation. Required for normal polarization of basal keratinocytes in skin, and for normal cell shape. Required for normal adhesion of keratinocytes to fibronectin and laminin, and for normal keratinocyte migration to wound sites. May mediate TGF-beta 1 signaling in tumor progression. Subcellular Location: Cytoplasm > cytoskeleton. Cell junction > focal adhesion. Cell projection > ruffle membrane. Constituent of focal adhesions. Localized at the basal aspect of skin keratinocytes, close to the cell membrane. Colocalizes with filamentous actin. Upon TGFB1 treatment, it localizes to membrane ruffles. Tissue Specificity: Expressed in brain, skeletal muscle, kidney, colon, adrenal gland, prostate, and placenta. Weakly or not expressed in heart, thymus, spleen, liver, small intestine, bone marrow, lung and peripheral blood leukocytes. Overexpressed in some colon and lung tumors. In skin, it is localized within the epidermis and particularly in basal keratocytes. Not detected in epidermal melanocytes and dermal fibroblasts. DISEASE: Defects in FERMT1 are the cause of Kindler syndrome (KINDS) [MIM:173650]. An autosomal recessive skin disorder characterized by skin blistering, photosensitivity, progressive poikiloderma, and extensive skin atrophy. Additional clinical features include gingival erosions, ocular, esophageal, gastrointestinal and urogenital involvement, and an increased risk of mucocutaneous malignancy. Note=Although most FERMT1 mutations are predicted to lead to premature termination of translation, and to loss of FERMT1 function, significant clinical variability is observed among patients. There is an association of FERMT1 missense and in-frame deletion mutations with milder disease phenotypes, and later onset of complications (PubMed:21936020). Similarity: Belongs to the kindlin family. Contains 1 FERM domain. Contains 1 PH domain. Database links: Entrez Gene: 55612 Human Entrez Gene: 241639 Mouse Omim: 607900 Human SwissProt: Q9BQL6 Human SwissProt: P59113 Mouse Unigene: 472054 Human Unigene: 209784 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Kindlin家族是新近發(fā)現(xiàn)的粘著斑蛋白（focal adhesion protein），有3個成員（Kindlin-1、Kindlin-2、Kindlin-3）。Kindlin家族參與整合素活化、細(xì)胞遷移、增殖和分化的調(diào)控，在臨床上與皮膚疾病發(fā)生、腫瘤的侵襲、心血管生成、免疫系統(tǒng)功能有密切關(guān)系。Kindlins異常可以導(dǎo)致多種遺傳性疾病，如Kindlin-1功能異常導(dǎo)致Kindler綜合征（Kindler syndrome，KS）和Kindlin-3功能異常導(dǎo)致白細(xì)胞黏附缺陷（1eukocyte adhesion deficiency，LAD—HI）。目前已在人類實體腫瘤（乳腺癌、前列腺癌、平滑肌肉瘤）中發(fā)現(xiàn)Kindlin-2與腫瘤的侵襲性及耐藥性有關(guān)。 |
