Lamin A + C R453W Antibody (12A-2F5) - BSA Free

Western Blot: Lamin A + C R453W Antibody (12A-2F5) [NBP1-77401] - Analysis of Lamin A + C R453W in HeLa cells transfected with Flag-tagged Lamin A 1) wild type, 2) R453W and 3) R482W.

• Reactivity: Hu
• Applications: WB, ELISA, ICC/IF, IP
• Clone: 12A-2F5
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated
• Format: BSA Free
• Concentration: 1 mg/ml

Lamin A + C R453W Antibody (12A-2F5) - BSA Free Summary

• Immunogen: Synthetic peptide corresponding to amino acids 449-457 of human Lamin A + C [Accession#: NP_733821.1]
• Specificity: Disease-associated point-mutant R453W of Lamin A + C
• Isotype: IgG1 Kappa
• Clonality: Monoclonal
• Host: Mouse
• Gene: LMNA
• Purity: Protein G purified

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 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).

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: Tris-Glycine and 0.15M NaCl
• Preservative: 0.05% Sodium Azide
• Concentration: 1 mg/ml
• Purity: Protein G purified

Alternate Names for Lamin A + C R453W Antibody (12A-2F5) - 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 R453W
• 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

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.

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 R453W Antibody (NBP1-77401)(1)

Publication using NBP1-77401

• 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] (WB, ICC/IF, IP, Human)

Review for Lamin A + C R453W Antibody (NBP1-77401) (1)

Average Rating: 4

(Based on 1 review)

We have 1 review tested in 1 species: Human.

reviewed by: Rajeshwari Bhat WB Human 06/01/2015

Summary

• Application: Western Blot
• Sample Tested: HeLa cells transfected with lamin A/C R453W
• Species: Human
• Lot: A1

Bioinformatics

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