Progerin

Progerin is a mutant version of the protein lamin A, which is involved in maintaining the structural integrity of the nucleus in cells. The production of progerin in the human body is associated with the premature aging disorder Hutchinson-Gilford Progeria Syndrome (HGPS).

Structure and Function

Progerin is a truncated version of lamin A, lacking 50 amino acids near one end of the protein. This alteration disrupts the protein's normal role in maintaining the structure of the nucleus, the cellular compartment that houses the majority of the cell's genetic material.

Role in Disease

The production of progerin is caused by a mutation in the LMNA gene, which provides instructions for making lamin A. This mutation is associated with HGPS, a disorder characterized by rapid aging that begins in childhood. People with HGPS typically live to their mid-teens to early twenties. It has been suggested that progerin may also play a role in the normal aging process.

Research and Treatment

Research into progerin has increased our understanding of HGPS and the aging process. Current treatments for HGPS are aimed at reducing the amount of progerin in cells or alleviating some of the symptoms of the disorder. However, there is currently no cure for HGPS.

See Also

• Lamin A
• Hutchinson-Gilford Progeria Syndrome
• LMNA

References

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Progerin

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  Biogenesis of lamin A in normal cells and the failure to generate mature lamin A in HGPS