Novel progerin-interactive partner proteins hnRNP E1, EGF, Mel 18, and UBC9 interact with lamin A/C.

The Hutchinson-Gilford progeria syndrome (HGPS or progeria) is an apparent accelerated aging disorder of childhood. Recently, HGPS has been characterized as one of a growing group of disorders known as laminopathies, which result from genetic defects of the lamin A/C (LMNA) gene. The majority of HGPS mutant alleles involve a ...
silent mutation, c.2063C>T resulting in G608G, that generates a cryptic splicing site in exon 11 of LMNA and consequently truncates 50 amino acids near the C-terminus of pre-lamin A/C. To explore possible mechanisms underlying the development of HGPS, we began a search for proteins that would uniquely interact with progerin (the truncated lamin A in HGPS) using a yeast two-hybrid system. Four new progerin interactive partner proteins were identified that had not been previously found to interact with lamin A/C: hnRNP E1, UBC9 (ubiquitin conjugating enzyme E2I), Mel-18, and EGF1. However, using control and progeria fibroblasts, co-immunoprecipitation studies of endogenous proteins did not show differential binding affinity compared to normal lamin A/C. Thus, we did not find evidence for uniquely interacting partner proteins using this approach, but did identify four new lamin A/C interactive partners.
Mesh Terms:
Cell Nucleus, Cells, Cultured, DNA-Binding Proteins, Epidermal Growth Factor, Fibroblasts, Heterogeneous-Nuclear Ribonucleoproteins, Humans, Lamin Type A, Lymphocytes, Progeria, Protein Binding, Protein Interaction Mapping, Repressor Proteins, Ubiquitin-Conjugating Enzymes
Biochem. Biophys. Res. Commun.
Date: Dec. 16, 2005
Download Curated Data For This Publication
66959
Switch View:
  • Interactions 14