Lamin A + C R482W Antibody (5H8-B4)

Novus Biologicals, part of Bio-Techne | Catalog # NBP1-77402

Novus Biologicals, part of Bio-Techne
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NBP1-77402
NBP1-77402SS
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Key Product Details

Species Reactivity

Validated:

Human

Cited:

Human

Applications

Validated:

ELISA, Immunocytochemistry/ Immunofluorescence, Immunoprecipitation, Western Blot

Cited:

Immunocytochemistry/ Immunofluorescence, Immunoprecipitation, Western Blot

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG1 kappa Clone # 5H8-B4

Concentration

This product is unpurified. The exact concentration of antibody is not quantifiable.

View all Lamin A + C R482W Antibodies »

Product Specifications

Immunogen

Hepatitis B Virus Capsid Antigen (HBcAg) Lamin A + C Ig-fold R482W fusion protein.

Specificity

Disease-associated point-mutant R482W of Lamin A + C

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1 kappa

Scientific Data Images for Lamin A + C R482W Antibody (5H8-B4)

Western Blot: Lamin A + C R482W Antibody (5H8-B4) [NBP1-77402]

Western Blot: Lamin A + C R482W Antibody (5H8-B4) [NBP1-77402]

Western Blot: Lamin A + C R482W Antibody (5H8-B4) [NBP1-77402] - Analysis of Lamin A + C R482W in HeLa cells transfected with Flag-tagged Lamin A 1) wild type, 2) R482W and 3) R453W.

Applications for Lamin A + C R482W Antibody (5H8-B4)

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 R482W (5H8-B4) 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

Unpurified

Formulation

Ascites

Preservative

0.1% Sodium Azide

Concentration

This product is unpurified. The exact concentration of antibody is not quantifiable.

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 R482W

In eukaryotic organisms, lamin A-type proteins i.e. lamin A/C are major components of lamina, a thin proteinaceous filamentous meshwork that underlies the inner nuclear membrane and besides regulating nuclear shape/rigidity, it also provide a scaffold-type support for chromosomes or replicating DNA to interact with epigenetic machinery. Lamin A/C gene (LMNA) mutations leads to neuromuscular and cardiac dystrophies, lipodystrophies, and premature ageing syndromes. In LMNA gene, missense mutations affecting two residues located within Ig-fold domain are common in laminopathy patients (i) an exchange of arginine 453 by tryptophan (R453W) detected in 16% of autosomal-dominant EDMD cases (11% laminopathy cases), (ii) the substitution of arginine 482 by either tryptophan, glutamine, or leucine (R482W/Q/L), which is responsible for more than 80% of FPLD (familial partial lipodystrophy - 13% laminopathy cases). FPLD is a disorder which results in abnormal fat distribution, insulin resistance, dyslipidemia and early cardiac heart disease. Our Lamin A+C R482W (5H8-B4) is a novel monoclonal antibody that specifically detects disease causing point mutants of A-type lamins namely FPLD associated lamin A/C R482W mutant (does not cross-react with wild-type counterpart) and was generated by immunization with a fusion protein consisting of the hepatitis B virus capsid protein, HBcAg, and the lamin A/C Ig-fold R482W domain inserted at the highly immunogenic viral c/e1 epitope. This antibody has been validated by Western blotting, competition experiments, IP, and ICC/IF microscopy assays, can be applied to selectively study features of the mutant A-type lamins in primary patient cells.

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 R82W, LAMIN C, LDP1, LFP, LGMD1B, LMN 1, LMN A, LMN C, LMN1, LMNA, LMNC, PRELAMIN A, PRO1, Progeria 1 (Hutchinson Gilford Type)

Entrez Gene IDs

4000 (Human)

Gene Symbol

LMNA

UniProt

NP_733821.1 (Human)

Additional Lamin A + C R482W Products

Product Documents for Lamin A + C R482W Antibody (5H8-B4)

Product Datasheets

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 R482W Antibody (5H8-B4)

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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