Anti-EVC2 antibody
英文名称 | EVC2 |
中文名称 | 膜蛋白EVC2抗体 |
别 名 | Ellis van Creveld syndrome 2; LBN; Limbin; LBN_HUMAN. |
DATASHEET
Host:Rabbit
Target Protein:EVC2
IR:Immunogen Range:101-200/1308
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:132884
Swiss Prot:Q86UK5
Source:KLH conjugated synthetic peptide derived from human EVC2:101-200/1308
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Background: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.
Size:100ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
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For research use only. Not intended for diagnostic or therapeutic use.
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