EVC2 antibody (AA 101-200)

Catalog No. ABIN719231

Product Details anti-EVC2 Antibody

Target: EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))

Binding Specificity: AA 101-200

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This EVC2 antibody is un-conjugated

Application: 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))

Predicted Reactivity: Human

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human EVC2

Isotype: IgG

Application Details

Application Notes: 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

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for EVC2

Target: EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))

Alternative Name: EVC2 (EVC2 Products)

Synonyms: EVC2 antibody, LBN antibody, 1110017L09Rik antibody, Lbn antibody, limbin antibody, EvC ciliary complex subunit 2 antibody, EVC2 antibody, Evc2 antibody

Background:

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.

Gene ID: 132884

Pathways: Hedgehog Signaling