Progeria, Accelerated Aging | Biochemical Mechanism of Progeria

The video explains progeria, a genetic disease causing accelerated aging, using Sam Burns as a case study. It details the role of lamin A in the nuclear lamina and how its improper processing leads to progeria. The video covers the biosynthetic pathway of lamin A, highlighting post-translational modifications and the consequences of a 50 amino acid deletion in progeria. This deletion results in progerin, which disrupts nuclear structure, leading to DNA damage and cellular senescence, ultimately causing accelerated aging.

What happens to the farnesyl group during the maturation of lamin A?

How does the mutation in progeria affect the cleavage of the protein?

Discuss the consequences of progerin incorporation into the nuclear lamina.