Anti-EVC1 antibody
| 英文名称 | EVC1 |
| 中文名称 | 软骨外胚层发育不良相关蛋白抗体 |
| 别 名 | Ellis van Creveld syndrome DWF 1; DWF1; Ellis van Creveld syndrome protein; Ellis-van Creveld syndrome; EVC; EVC1; EVC 1; EVC-1; EVCL; MGC105107; EVC_HUMAN. |
DATASHEET
Host:Rabbit
Target Protein:EVC1
IR:Immunogen Range:251-350/408
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:2121
Swiss Prot:P57679
Source:KLH conjugated synthetic peptide derived from human EVC1:251-350/408
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:EVC is an autosomal skeletal dysplasia caused by mutations in the EVC and EVC2 genes. Found in developing ribs, heart, kidney and lung, the EVC gene is responsible for normal development of the face, limbs, teeth and nails. The protein expressed by the EVC gene is an intracellular component of the hedgehog signal pathway that contains a leucine zipper and transmembrane domain. Defects in the EVC gene can lead to short-limb dwarfism, ectodermal dysplasia and cardiac anomalies such as irregular atrioventricular septum development. Additionally, the EVC gene has been implicated in Weyers acrodental dysostosis, an autosomal dominant disease characterized by facial abnormalities and limb defects.
Size:100ul
Concentration:1mg/ml
Applications: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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