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

Biochem Biophys Res Commun. 2005 Dec 16;338(2):855-61. doi: 10.1016/j.bbrc.2005.10.020. Epub 2005 Oct 14.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Nucleus / metabolism*
  • Cells, Cultured
  • DNA-Binding Proteins / metabolism*
  • Epidermal Growth Factor / metabolism*
  • Fibroblasts / metabolism
  • Heterogeneous-Nuclear Ribonucleoproteins / metabolism*
  • Humans
  • Lamin Type A / metabolism*
  • Lymphocytes / metabolism
  • Polycomb Repressive Complex 1
  • Progeria / metabolism*
  • Progeria / pathology
  • Protein Binding
  • Protein Interaction Mapping
  • RNA-Binding Proteins
  • Repressor Proteins
  • Ubiquitin-Conjugating Enzymes / metabolism*


  • DNA-Binding Proteins
  • Heterogeneous-Nuclear Ribonucleoproteins
  • Lamin Type A
  • PCBP1 protein, human
  • PCGF2 protein, human
  • RNA-Binding Proteins
  • Repressor Proteins
  • lamin C
  • Epidermal Growth Factor
  • Ubiquitin-Conjugating Enzymes
  • Polycomb Repressive Complex 1
  • ubiquitin-conjugating enzyme UBC9

Associated data

  • OMIM/115200
  • OMIM/159001
  • OMIM/181350