Abcam, ab66587, Anti-Progerin antibody [13A4]

Size: 100µg
Mouse Monoclonal Lamin-A/C antibody. Suitable for WB and reacts with Human samples. Cited in 22 publications.
Key facts
Host species:Mouse,
Clonality:Monoclonal,
Clone number:13A4,
Isotype:IgG1,
Light chain type:kappa,
Carrier free:No,
Reacts with:Human,
Applications:WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:The exact immunogen used to generate this antibody is proprietary information.

Product details:
Progerin is expressed as the result of a de novo point mutation in the lamin A gene and is the underlying cause of Hutchison-Gilford progeria syndrome (accelerated aging). There is now also evidence that small amounts of Progerin are also produced in normal cells and that this might have a link with the normal aging process (Cao et al., PNAS, 2007 Mar 20;104(12):4949-54.). Gly608Gly is the most frequent HGPS-associated mutation. It is a silent base substitution that activates a cryptic splice donor in exon 11 of LMNA (BOX 3). Use of this anomalous splice donor leads to the loss of 150 nucleotides from the 3' end of exon 11 in the mature lamin A mRNA, and internal deletion of 50 amino-acid residues from the C terminus of lamin A. Progerin is the resulting mutant protein. Progerin retains its C-terminal CAAX motif, and therefore is farnesylated.
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Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification, Storage buffer-pH: 7.4Preservative: 0.02% Sodium azideConstituents: PBS, 6.97% L-Arginine, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Progerin also known as a truncated version of Lamin A (LMNA) is a mutant protein that emerges due to a cryptic splice site in the LMNA gene. This results in a 607 amino acid protein with a mass of approximately 65 kDa. Progerin is expressed in the cell nucleus and accumulates over time predominantly affecting cells with high turnover rates such as those in the skin cardiovascular system and adipose tissues. This abnormal protein disrupts the normal function of the nuclear lamina.
Biological function summary
Progerin acts by compromising cellular structural integrity and altering gene expression. It integrates into the nuclear lamina but fails to undergo proper post-translational modification which is vital for cellular functions like mitosis nuclear migration and chromatin organization. Progerin's presence affects the mechanical properties of the nucleus hindering its role in stress response and inducing cellular aging. This mutant protein functions independently as it does not form part of a larger protein complex but rather disturbs the assembly of the lamina network.
Pathways
Progerin influences the mechanistic pathways responsible for cellular aging and apoptosis. It disrupts the Wnt/β-catenin pathway affecting cell cycle regulation and the MAPK signaling pathway which relates to stress and growth responses. Progerin shares a close association with prelamin A and Lamin C as all these proteins derive from the same LMNA gene and interact through shared pathways influencing nuclear shape and transcriptome stability.
Progerin plays a significant role in Hutchinson-Gilford Progeria Syndrome (HGPS) and is implicated in age-related disorders like arteriosclerosis. HGPS results from the accumulation of Progerin leading to premature aging due to the faulty nuclear architecture. Progerin's relation with other proteins such as Lamin B1 and Lamin C affects the nuclear envelope's integrity exacerbating the progression of these disorders. The presence of Progerin may serve as a potential target for therapeutic interventions aimed at blocking its production or function which could mitigate its effects on aging-related pathologies.