Cell & Bioscience (Jun 2012)

From loss to gain: role for SUN1 in laminopathies

  • Liu Baohua,
  • Jin Dong-Yan,
  • Zhou Zhongjun

DOI
https://doi.org/10.1186/2045-3701-2-21
Journal volume & issue
Vol. 2, no. 1
p. 21

Abstract

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Abstract Mutations in LMNA encoding lamins A and C are associated with at least 10 different degenerative disorders affecting diverse tissues, collectively called laminopathies. A recent study showed that mis-accumulation of SUN1 underlies the pathology of degenerative features in laminopathies, and concomitantly suggests a gain-of-function versus a loss-of-function model for the action of lamin A mutants.