Requirements for efficient proteolytic cleavage of prelamin A by ZMPSTE24.

ABSTRACT:

Background

The proteolytic maturation of the nuclear protein lamin A by the zinc metalloprotease ZMPSTE24 is critical for human health. The lamin A precursor, prelamin A, undergoes a multi-step maturation process that includes CAAX processing (farnesylation, proteolysis and carboxylmethylation of the C-terminal CAAX motif), followed by ZMPSTE24-mediated cleavage of the last 15 amino acids, including the modified C-terminus. Failure to cleave the prelamin A "tail", due to mutations in either prelamin A or ZMPSTE24, results in a permanently prenylated form of prelamin A that underlies the premature aging disease Hutchinson-Gilford Progeria Syndrome (HGPS) and related progeroid disorders.

Methodology/principal findings

Here we have investigated the features of the prelamin A su

SUBMITTER: Barrowman J 

PROVIDER: S-EPMC3280227 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature