Lamin A Progeria Mouse Model

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货号 : EDMM001

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产品属性

Product Name Lamin A Progeria Mouse Model
Strain Name C57BL/6J-LmnaTm1/MC
Advantages 1. Short lifespan
2. Distinct progeria phenotype
3. High consistency with defined genetic background
4. Suitable for various aging-related studies
Application 1. Mechanistic research on aging-related diseases
2. Development of anti-aging therapeutics and products
Nomenclature C57B/6J-LmnaTm1/MCL

Modeling Principle

This progeria mouse model is based on a point mutation in the Lamin A gene that activates a cryptic splice site in exon 11 (G608G: GGC→GGT). This leads to the deletion of 50 amino acids from the C-terminus of the prelamin A polypeptide. The resulting truncated protein is known as progerin. Progerin retains the CXXF motif but lacks the ZMPSTE24 cleavage site, preventing the normal post-translational processing at the C-terminus. The exposed farnesylated cysteine methyl ester remains permanently attached. Over time, the accumulation of progerin causes various cellular defects and dysfunctions, ultimately leading to aging of cells and tissues.

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