获取电话Quantity size:
0.2ml
Concentration:
1mg/ml 软骨外胚层发育不良相关蛋白抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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软骨外胚层发育不良相关蛋白抗体 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.
软骨外胚层发育不良相关蛋白抗体Also known as:
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.
Specificity:
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Rabbit Polyclonal IgG,软骨外胚层发育不良相关蛋白抗体 affinity purified by Protein A.
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Reacts with: Human, .
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Immunogen: KLH conjugated synthetic peptide derived from human EVC1.
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Predicted Molecular Weight: 112kDa.
软骨外胚层发育不良相关蛋白抗体Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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