膜蛋白EVC2抗体

英文名称  Anti-EVC2 

中文名称  膜蛋白EVC2抗体 

膜蛋白EVC2抗体别    名  Ellis van Creveld syndrome 2; LBN; Limbin; LBN_HUMAN. 

浓    度  1mg/1ml 

规 格  0.1ml/100μg  0.2ml/200μg     

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human

产品类型  一抗    

公司全程供应Abcam、CST、Abnova、BD、GeneTex、Proteintech、SB、Abgent、AbFrontier、AnaSpec、Agrisera、Alomone、Epitomics、Active Motif、Assaybiotech、Biorbyt、BioVision、Bender、CaymanChemical、Covance、ChromoTek、Enzo、eBioscience、Glen Research、MBL、ProSci、PeproTech、Swant、Vector等品牌公司抗体，产品货期短、膜蛋白EVC2抗体品质保证、全国免邮，并提供抗体相应实验技术指导。

研究领域  细胞生物 细胞膜蛋白  

蛋白分子量  predicted molecular weight: 145kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human EVC2 (116-165aa) 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  Flow-Cyt=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.  

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. 

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

产品介绍 EVC2 is an integral membrane protein that plays a vital role in bone formation and skeletal development. Defects in EVC2 are a cause of Ellis-van Creveld syndrome (EVC), also known as chondroectodermal dysplasia. EVC is an autosomal recessive disorder characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia and cardiac anomalies. Patients manifest short-limb dwarfism, short ribs, postaxial polydactyly and dysplastic nails and teeth. Congenital heart defects, most commonly an atrioventricular septal defect, are observed in 60% of affected individuals.

膜蛋白EVC2抗体Function : Positive regulator of the hedgehog signaling pathway. Plays a critical role in bone formation and skeletal development.

Subunit : Interacts with EVC.

Subcellular Location : Cell membrane; Single-pass type I membrane protein. Cytoplasm, cytoskeleton, cilium basal body. Cell projection, cilium. Cell projection, cilium membrane. Nucleus.

Tissue Specificity : Found in the heart, placenta, lung, liver, skeletal muscle, kidney and pancreas.

DISEASE : Defects in EVC2 are a cause of Ellis-van Creveld syndrome (EVC) [MIM:225500]; also known as chondroectodermal dysplasia. EVC is an autosomal recessive disorder characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia and cardiac anomalies. Patients manifest short-limb dwarfism, short ribs, postaxial polydactyly and dysplastic nails and teeth. Congenital heart defects, most commonly an atrioventricular septal defect, are observed in 60% of affected individuals.

Defects in EVC2 are a cause of acrofacial dysostosis Weyers type (WAD) [MIM:193530]; also known as Curry-Hall syndrome. Acrofacial dysostoses are a heterogeneous group of disorders combining limb defects with facial abnormalities. WAD is an autosomal dominant disorder characterized by dysplastic nails, postaxial polydactyly, acrofacial dysostosis, short limbs and short stature. The phenotype is milder than Ellis-van Creveld syndrome.

Database links : UniProtKB/Swiss-Prot: Q86UK5.1

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