EVC2 anticorps (AA 101-200)
Aperçu rapide pour EVC2 anticorps (AA 101-200) (ABIN719231)
Antigène
Voir toutes EVC2 AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Épitope
- AA 101-200
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Homologie
- Human
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Purification
- Purified by Protein A.
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Immunogène
- KLH conjugated synthetic peptide derived from human EVC2
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Isotype
- IgG
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Indications d'application
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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 -
Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 1 μg/μL
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Buffer
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
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Agent conservateur
- ProClin
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Précaution d'utilisation
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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Stock
- 4 °C,-20 °C
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Stockage commentaire
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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Date de péremption
- 12 months
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- EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))
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Autre désignation
- EVC2
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Sujet
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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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ID gène
- 132884
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Pathways
- Signalisation Hedgehog
Antigène
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