Lamin A + C R453W Antibody (12A-2F5) [mFluor Violet 610 SE] 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 |
Innovator's Reward |
Test in a species/application not listed above to receive a full credit towards a future purchase. |
Applications/Dilutions
Dilutions |
- ELISA
- Immunocytochemistry/ Immunofluorescence
- Immunoprecipitation
- Western Blot
|
Application Notes |
Optimal dilution of this antibody should be experimentally determined. |
Packaging, Storage & Formulations
Storage |
Store at 4C in the dark. |
Buffer |
50mM Sodium Borate |
Preservative |
0.05% Sodium Azide |
Purity |
Protein G purified |
Notes
mFluor(TM) is a trademark of AAT Bioquest, Inc. 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.
Alternate Names for Lamin A + C R453W Antibody (12A-2F5) [mFluor Violet 610 SE]
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.
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