产品货号 :  mlR17063

英文名称 :  Kindlin

中文名称 :  整合素相互作用蛋白Kindlin抗体

别    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.  

研究领域 :  肿瘤  细胞生物  肿瘤细胞生物标志物  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :   Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, Sheep,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.

分 子 量 :  77kDa

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human Kindlin:601-677/677

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  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.

PubMed :  PubMed

产品介绍 :   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.

SWISS:

Q9BQL6

Gene ID:

55612

Important Note:

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

Kindlin家族是新近发现的粘着斑蛋白（focal adhesion protein），有3个成员（Kindlin-1、Kindlin-2、Kindlin-3）。Kindlin家族参与整合素活化、细胞迁移、增殖和分化的调控，在临床上与皮肤疾病发生、肿瘤的侵袭、心血管生成、免疫系统功能有密切关系。Kindlins异常可以导致多种遗传性疾病，如Kindlin-1功能异常导致Kindler综合征（Kindler syndrome，KS）和Kindlin-3功能异常导致白细胞黏附缺陷（1eukocyte adhesion deficiency，LAD—HI）。目前已在人类实体肿瘤（乳腺癌、前列腺癌、平滑肌肉瘤）中发现Kindlin-2与肿瘤的侵袭性及耐药性有关。

产品图片