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Lamin A + C R453W Antibody (12A-2F5) [Alexa Fluor® 647]

Novus Biologicals, part of Bio-Techne | Catalog # NBP1-77401AF647

Novus Biologicals, part of Bio-Techne

Key Product Details

Species Reactivity

Validated:

Human

Applications

ELISA, Immunocytochemistry/ Immunofluorescence, Immunoprecipitation, Western Blot

Label

Alexa Fluor 647 (Excitation = 650 nm, Emission = 668 nm)

Antibody Source

Monoclonal Mouse IgG1 kappa Clone # 12A-2F5

Concentration

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

Product Summary for Lamin A + C R453W Antibody (12A-2F5) [Alexa Fluor® 647]

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) [Alexa Fluor® 647]

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.
Please Note: Optimal dilutions 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) [Alexa Fluor® 647]

Certificate of Analysis

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

Product Specific Notices for Lamin A + C R453W Antibody (12A-2F5) [Alexa Fluor® 647]

Alexa Fluor (R) products are provided under an intellectual property license from Life Technologies Corporation. The purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: (i) in manufacturing; (ii) to provide a service, information, or data in return for payment; (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5791 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@lifetech.com. This conjugate is made on demand. Actual recovery may vary from the stated volume of this product. The volume will be greater than or equal to the unit size stated on the datasheet.

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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