EVC2 抗体 (AA 101-200)
Quick Overview for EVC2 抗体 (AA 101-200) (ABIN719231)
抗原
See all EVC2 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 101-200
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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 EVC2
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亚型
- IgG
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anti-Ellis Van Creveld Syndrome 2 (EVC2) (AA 30-199) antibody
EVC2 适用: 人 ELISA, IHC, IF 宿主: 兔 Polyclonal unconjugated
anti-Ellis Van Creveld Syndrome 2 (EVC2) (C-Term) antibodyEVC2 适用: 人 WB, IF, ICC 宿主: 兔 Polyclonal unconjugated
anti-Ellis Van Creveld Syndrome 2 (EVC2) (C-Term) antibodyVerified EVC2 适用: 人 ELISA, IHC 宿主: 山羊 Polyclonal unconjugated
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应用备注
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WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500 -
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
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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
- 4 °C,-20 °C
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储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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有效期
- 12 months
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- EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))
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别名
- EVC2
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背景
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Synonyms: Ellis van Creveld syndrome 2, LBN, Limbin, LBN_HUMAN.
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.
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基因ID
- 132884
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途径
- Hedgehog Signaling
抗原
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