Nesprin-2 mediated nuclear trafficking and its clinical implications

Nucleus. 2015;6(6):479-89. doi: 10.1080/19491034.2015.1128608. Epub 2015 Dec 8.

Abstract

Nuclear translocation of proteins has a crucial role in the pathogenesis of cancer, Alzheimer disease and viral infections. A complete understanding of nuclear trafficking mechanisms is therefore necessary in order to establish effective intervention strategies. Here we elucidate the role of Nesprin-2 in Ca(2+)/Calmodulin mediated nuclear transport. Nesprin-2 is an actin-binding nuclear envelope (NE) protein with roles in maintaining nuclear structure and location, regulation of transcription and mechanotransduction. Upon depletion of Nesprin-2 using shRNA, HaCaT cells show abnormal localization of the shuttling proteins BRCA1 and NF-κB. We show that their nuclear transport is unlikely due to the canonical RAN mediated nuclear import, but rather to a RAN independent Ca(2+)/Calmodulin driven mechanism involving Nesprin-2. We report novel interactions between the actin-binding domain of Nesprin-2 and Calmodulin and between the NLS containing region of BRCA1 and Calmodulin. Strikingly, displacing Nesprins from the NE resulted in increased steady state Ca(2+) concentrations in the cytoplasm suggesting a previously unidentified role of Nesprins in Ca(2+) regulation. On comparing Nesprin-2 and BRCA1 localization in the ovarian cancer cell lines SKOV-3 and Caov-3, Nesprin-2 and BRCA1 were localized to the NE envelope and the nucleus in SKOV-3, respectively, and to the cytoplasm in Caov-3 cells. Fibroblasts obtained from EDMD5 (Emery Dreifuss muscular dystrophy) patients showed loss of Nesprin-2 from the nuclear envelope, corresponding reduced nuclear localization of BRCA1 and enhanced cytoplasmic Ca(2+). Taken together, the data suggests a novel role of Nesprin-2 in Ca(2+)/Calmodulin mediated nuclear trafficking and provides new insights which can guide future therapies.

Keywords: BRCA1; Calmodulin; NF-κB; Nesprin-2; RAN; nuclear transport.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Active Transport, Cell Nucleus / physiology*
  • Animals
  • BRCA1 Protein / metabolism
  • COS Cells
  • Calcium / metabolism
  • Calmodulin / metabolism
  • Cell Line
  • Chlorocebus aethiops
  • Genes, Reporter
  • Humans
  • Membrane Proteins / metabolism
  • Microfilament Proteins / antagonists & inhibitors
  • Microfilament Proteins / genetics
  • Microfilament Proteins / metabolism*
  • Microscopy, Confocal
  • Muscular Dystrophy, Emery-Dreifuss / metabolism
  • Muscular Dystrophy, Emery-Dreifuss / pathology
  • NF-kappa B / metabolism
  • Nerve Tissue Proteins / antagonists & inhibitors
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism*
  • Nuclear Pore / metabolism
  • Nuclear Proteins / antagonists & inhibitors
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism*
  • Plasmids / genetics
  • Plasmids / metabolism
  • RNA Interference
  • RNA, Small Interfering / metabolism
  • ran GTP-Binding Protein / metabolism

Substances

  • BRCA1 Protein
  • Calmodulin
  • Membrane Proteins
  • Microfilament Proteins
  • NF-kappa B
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • RNA, Small Interfering
  • SYNE2 protein, human
  • emerin
  • ran GTP-Binding Protein
  • Calcium