Abcam, ab320642, Anti-Progerin antibody [EPR28694-72]

Size: 20µL / 100µL / 1mL
Rabbit Recombinant Monoclonal Lamin-A/C antibody. Suitable for WB, IHC-P, ICC/IF, IP and reacts with Transfected cell lysate - Human, Transfected cell line - Human samples.
Key facts
Host species:Rabbit,
Clonality:Monoclonal,
Clone number:EPR28694-72,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:ICC/IF, IP, WB, IHC-PSee 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:
Patented technology
Our RabMAb
technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to
RabMAb® patents
What are the advantages of a recombinant monoclonal antibody?
This product is a recombinant monoclonal antibody, which offers several advantages including:
- High batch-to-batch consistency and reproducibility
- Improved sensitivity and specificity
- Long-term security of supply
- Animal-free batch production
For more information, read more on
recombinant antibodies

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.2 - 7.4Preservative: 0.01% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 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.