EVC2 抗体 (AA 379-901)
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Quick Overview for EVC2 抗体 (AA 379-901) (ABIN7874490)
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 379-901
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原理
- EVC2 Antibody / Ellis-van Creveld syndrome protein 2
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纯化方法
- Immunogen affinity purified
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免疫原
- E.coli-derived mouse EVC2 recombinant protein (Position: Q379-D901) was used as the immunogen for the EVC2 antibody.
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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
- Optimal dilution of the EVC2 antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
- 4 °C,-20 °C
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储存方法
- After reconstitution, the EVC2 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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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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EVC2 antibody detects Ellis-van Creveld syndrome protein 2, a ciliary basal body protein required for Hedgehog signaling and skeletal development. The UniProt recommended name is Ellis-van Creveld syndrome protein 2 (EVC2). Also known as Limbin, this protein anchors signaling complexes at the base of the primary cilium, coordinating receptor localization and developmental signal transduction.
Functionally, EVC2 antibody identifies a 1,285-amino-acid transmembrane protein localized to the ciliary pocket region. EVC2 forms a complex with EVC, tethering Hedgehog pathway components such as Smoothened and Gli transcription factors at the cilium base to modulate downstream transcriptional activity. It is essential for skeletal morphogenesis and growth plate function.
The EVC2 gene is located on chromosome 4p16.2 and is expressed in ciliated tissues, including cartilage, bone, and embryonic organs. Proper EVC2 function maintains ciliary integrity and ensures controlled Hedgehog pathway activation during tissue differentiation.
Pathologically, mutations in EVC2 cause Ellis-van Creveld syndrome and Weyers acrofacial dysostosis, characterized by short stature, polydactyly, and craniofacial abnormalities. Disruption of EVC2 impairs Hedgehog signaling, leading to developmental malformations. Research using EVC2 antibody supports studies in ciliopathies, developmental biology, and skeletal genetics.
EVC2 antibody is validated for western blotting, immunofluorescence, and immunohistochemistry to detect ciliary and developmental proteins. NSJ Bioreagents provides EVC2 antibody reagents optimized for cilia structure, signaling, and morphogenesis research.
Structurally, Ellis-van Creveld syndrome protein 2 is a membrane-tethered scaffold with coiled-coil domains that mediate interaction with EVC and ciliary membrane components. This antibody enables examination of EVC2's function in Hedgehog signaling and developmental regulation. -
UniProt
- Q8K1G2
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途径
- Hedgehog Signaling
抗原
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