Drugs affecting prelamin A processing: effects on heterochromatin organization

Exp Cell Res. 2008 Feb 1;314(3):453-62. doi: 10.1016/j.yexcr.2007.11.012. Epub 2007 Nov 24.

Abstract

Increasing interest in drugs acting on prelamin A has derived from the finding of prelamin A involvement in severe laminopathies. Amelioration of the nuclear morphology by inhibitors of prelamin A farnesylation has been widely reported in progeroid laminopathies. We investigated the effects on chromatin organization of two drugs inhibiting prelamin A processing by an ultrastructural and biochemical approach. The farnesyltransferase inhibitor FTI-277 and the non-peptidomimetic drug N-acetyl-S-farnesyl-l-cysteine methylester (AFCMe) were administered to cultured control human fibroblasts for 6 or 18 h. FTI-277 interferes with protein farnesylation causing accumulation of non-farnesylated prelamin A, while AFCMe impairs the last cleavage of the lamin A precursor and is expected to accumulate farnesylated prelamin A. FTI-277 caused redistribution of heterochromatin domains at the nuclear interior, while AFCMe caused loss of heterochromatin domains, increase of nuclear size and nuclear lamina thickening. At the biochemical level, heterochromatin-associated proteins and LAP2 alpha were clustered at the nuclear interior following FTI-277 treatment, while they were unevenly distributed or absent in AFCMe-treated nuclei. The reported effects show that chromatin is an immediate target of FTI-277 and AFCMe and that dramatic remodeling of chromatin domains occurs following treatment with the drugs. These effects appear to depend, at least in part, on the accumulation of prelamin A forms, since impairment of prelamin A accumulation, here obtained by 5-azadeoxycytidine treatment, abolishes the chromatin effects. These results may be used to evaluate downstream effects of FTIs or other prelamin A inhibitors potentially useful for the therapy of laminopathies.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acetylcysteine / analogs & derivatives
  • Acetylcysteine / pharmacology
  • Adult
  • Antimetabolites, Antineoplastic / pharmacology
  • Azacitidine / analogs & derivatives
  • Azacitidine / pharmacology
  • Cell Nucleus / drug effects*
  • Cell Nucleus / metabolism
  • Cell Nucleus / ultrastructure
  • Cells, Cultured
  • Chromatin Assembly and Disassembly / drug effects*
  • Chromatin Assembly and Disassembly / genetics
  • DNA-Binding Proteins / drug effects
  • DNA-Binding Proteins / metabolism
  • Decitabine
  • Enzyme Inhibitors / pharmacology
  • Farnesyltranstransferase / antagonists & inhibitors*
  • Farnesyltranstransferase / metabolism
  • Fibroblasts / drug effects
  • Fibroblasts / metabolism
  • Heterochromatin / drug effects*
  • Heterochromatin / genetics
  • Heterochromatin / ultrastructure
  • Humans
  • Lamin Type A / drug effects
  • Lamin Type A / metabolism
  • Membrane Proteins / drug effects
  • Membrane Proteins / metabolism
  • Methionine / analogs & derivatives
  • Methionine / pharmacology
  • Nuclear Lamina / drug effects
  • Nuclear Lamina / metabolism
  • Nuclear Lamina / ultrastructure
  • Nuclear Proteins / drug effects*
  • Nuclear Proteins / metabolism
  • Protein Precursors / drug effects*
  • Protein Precursors / metabolism
  • Protein Prenylation / drug effects*
  • Protein Prenylation / physiology

Substances

  • Antimetabolites, Antineoplastic
  • DNA-Binding Proteins
  • Enzyme Inhibitors
  • FTI 277
  • Heterochromatin
  • Lamin Type A
  • Membrane Proteins
  • Nuclear Proteins
  • Protein Precursors
  • lamina-associated polypeptide 2
  • prelamin A
  • Decitabine
  • Methionine
  • Farnesyltranstransferase
  • N-acetyl-S-farnesylcysteine
  • Azacitidine
  • Acetylcysteine