Key Product Details

Species Reactivity

Human

Applications

ELISA, Immunocytochemistry/ Immunofluorescence, Immunoprecipitation, Western Blot

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG1 kappa Clone # 12A-2F5

Format

Azide and BSA Free

Concentration

1 mg/ml

View all Lamin A + C R453W Antibodies »

Product Specifications

Immunogen

Synthetic peptide corresponding to amino acids 449-457 of human Lamin A + C

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1 kappa

Scientific Data Images for Lamin A + C R453W Antibody (12A-2F5) - Azide and BSA Free

Western Blot: Lamin A + C R453W Antibody (12A-2F5)Azide and BSA Free [NBP2-80823]

Western Blot: Lamin A + C R453W Antibody (12A-2F5)Azide and BSA Free [NBP2-80823]

Western Blot: Lamin A + C R453W Antibody (12A-2F5) - Azide and BSA Free [NBP2-80823] - Analysis of Lamin A + C R453W in HeLa cells transfected with Flag-tagged Lamin A 1) wild type, 2) R453W and 3) R482W. Image from the standard format of this antibody.

Applications for Lamin A + C R453W Antibody (12A-2F5) - Azide and BSA Free

Application
Recommended Usage

ELISA

1:100-1:2000

Immunocytochemistry/ Immunofluorescence

1:10-1:2000

Immunoprecipitation

1:10-1:500

Western Blot

1:1000
Application Notes
This Lamin A + C R453W (12A-2F5) point-mutant specific antibody is useful for Western blot, ELISA, Immunoprecipitation and Immunocytochemistry/Immunofluorescence. In WB bands can be seen at 74kDa (Lamin A) and 65 kDa (Lamin C).

Formulation, Preparation, and Storage

Purification

Protein G purified

Formulation

Tris-Glycine, 0.15 M NaCl

Format

Azide and BSA Free

Preservative

No Preservative

Concentration

1 mg/ml

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.

Background: Lamin A + C R453W

The lamin A gene is important in making nuclear structure and substructures for DNA replication appropriately in cells. The R453W mutation of LMNA, however, negates much of these normal characteristics, and instead causes the phenotypes of Emery-Dreifuss muscular dystrophy (EDMD). The mutation impairs myoblast formation and differentiation, and contributes to muscle wasting. R453W lamin changes the location of the H2K27me3 modified histone interaction with chromatin, which inactivates certain downstream genes. It also affects the normal lamin function of scaffolding substrates for the MEK-ERK pathway, and disrupts the structure of other binding sites. Thus, R453W seems to cause phenotypes of muscular dystrophy both through direct genetic impact, but also through epigenetic influence over normal scaffolding functionality related to DNA replication.

Alternate Names

70 kDa Lamin, CDCD1, CDDC, Charcot Marie Tooth Disease Axonal Type 2B1, CMD1A, CMT2B1, EMD2, FPL, FPLD, HGPS, IDC, LAMIN A, Lamin A/C, Lamin A/C R453W, LAMIN C, LDP1, LFP, LGMD1B, LMN 1, LMN A, LMN C, LMN1, LMNA, LMNC, PRELAMIN A, PRO1, Progeria 1 (Hutchinson Gilford Type)

Gene Symbol

LMNA

Additional Lamin A + C R453W Products

Product Documents for Lamin A + C R453W Antibody (12A-2F5) - Azide and BSA Free

Product Datasheets

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Download SDS

Product Specific Notices for Lamin A + C R453W Antibody (12A-2F5) - Azide and BSA Free

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Privacy and Cookie Policy
Site Map
© Copyright 2024 Bio-Techne. All Rights Reserved.