EVC2 抗体 (AA 101-200)
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- 抗原 See all EVC2 抗体
- EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))
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抗原表位
- AA 101-200
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适用
- 人
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This EVC2 antibody is un-conjugated
- 应用范围
- ELISA, Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
- 预测反应
- Human
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human EVC2
- 亚型
- IgG
- Top Product
- Discover our top product EVC2 Primary Antibody
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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
- 浓度
- 1 μg/μL
- 缓冲液
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- 4 °C,-20 °C
- 储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
- 有效期
- 12 months
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- 抗原
- EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))
- 别名
- EVC2 (EVC2 产品)
- 别名
- EVC2 antibody, LBN antibody, 1110017L09Rik antibody, Lbn antibody, limbin antibody, EvC ciliary complex subunit 2 antibody, EVC2 antibody, Evc2 antibody
- 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.
- 基因ID
- 132884
- 途径
- Hedgehog Signaling
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