EVC 抗体 (AA 251-350) (Cy5)
Quick Overview for EVC 抗体 (AA 251-350) (Cy5) (ABIN1408234)
抗原
See all EVC 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 251-350
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预测反应
- Human
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human EVC1
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亚型
- IgG
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anti-Ellis Van Creveld Syndrome (EVC) (AA 1-535) antibody
EVC 适用: 人 WB 宿主: 小鼠 Polyclonal unconjugated
anti-Ellis Van Creveld Syndrome (EVC) (AA 493-602) antibodyEVC 适用: 人 ELISA, WB, IHC 宿主: 兔 Polyclonal unconjugated
anti-Ellis Van Creveld Syndrome (EVC) (AA 493-602) antibodyEVC 适用: 人 ELISA, WB 宿主: 小鼠 Monoclonal 3C4 unconjugated
anti-Ellis Van Creveld Syndrome (EVC) (AA 493-602) antibodyEVC 适用: 人 ELISA, WB 宿主: 小鼠 Polyclonal unconjugated
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应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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- EVC (Ellis Van Creveld Syndrome (EVC))
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别名
- EVC1
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背景
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Synonyms: 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.
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.
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途径
- Hedgehog Signaling
抗原
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