Lamin A + C R453W Antibody (12A-2F5) [Janelia Fluor® 549]

Novus Biologicals, part of Bio-Techne | Catalog # NBP1-77401JF549

Novus Biologicals, part of Bio-Techne

Key Product Details

Species Reactivity

Human

Applications

ELISA, Immunocytochemistry/ Immunofluorescence, Immunoprecipitation, Western Blot

Label

Janelia Fluor 549

Antibody Source

Monoclonal Mouse IgG1 kappa Clone # 12A-2F5

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

View all Lamin A + C R453W Antibodies »

Product Specifications

Immunogen

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

Specificity

Disease-associated point-mutant R453W of Lamin A + C

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1 kappa

Applications for Lamin A + C R453W Antibody (12A-2F5) [Janelia Fluor® 549]

Application
Recommended Usage

ELISA

Optimal dilutions of this antibody should be experimentally determined.

Immunocytochemistry/ Immunofluorescence

Optimal dilutions of this antibody should be experimentally determined.

Immunoprecipitation

Optimal dilutions of this antibody should be experimentally determined.

Western Blot

Optimal dilutions of this antibody should be experimentally determined.
Application Notes
Optimal dilution of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Purification

Protein G purified

Formulation

50mM Sodium Borate

Preservative

0.05% Sodium Azide

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

Shipping

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

Stability & Storage

Store at 4C in the dark.

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) [Janelia Fluor® 549]

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) [Janelia Fluor® 549]

Sold under license from the Howard Hughes Medical Institute, Janelia Research Campus.

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.

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