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

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.

• Reactivity: Hu
• Applications: WB, ELISA, ICC/IF, IP
• Clone: 5H8-B4
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated
• Format: BSA Free

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

• 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
• Isotype: IgG1 Kappa
• Clonality: Monoclonal
• Host: Mouse
• Gene: LMNA
• Purity: Unpurified

Applications/Dilutions

• Dilutions:
  • 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).

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: Ascites
• Preservative: 0.1% Sodium Azide
• Purity: Unpurified

Alternate Names for Lamin A + C R482W Antibody (5H8-B4) - BSA Free

• 70 kDa Lamin
• CDCD1
• CDDC
• Charcot Marie Tooth Disease Axonal Type 2B1
• CMD1A
• CMT2B1
• EMD2
• FPL
• FPLD
• HGPS
• IDC
• LAMIN A
• Lamin A/C R82W
• Lamin A/C
• LAMIN C
• LDP1
• LFP
• LGMD1B
• LMN 1
• LMN A
• LMN C
• LMN1
• LMNA
• LMNC
• PRELAMIN A
• PRO1
• Progeria 1 (Hutchinson Gilford Type)

Background

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.

Limitations

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.

Publications for Lamin A + C R482W Antibody (NBP1-77402)(1)

Publication using NBP1-77402

• Roblek M, Schuchner S, Huber V, Ollram K, Vlcek-Vesely S, Foisner R, Wehnert M, Ogris E. Monoclonal antibodies specific for disease-associated point-mutants: lamin A/C R453W and R482W. PLoS One. 5(5):e10604. 2010-05-13 [PMID: 20498701] (ICC/IF, IP, WB, Human)

Bioinformatics

• Gene Symbol: LMNA
• Entrez:
  • 4000(Human)
• Uniprot:
  • NP_733821.1(Human)