Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Comment
. 2012 Sep 11;22(17):R678-80.
doi: 10.1016/j.cub.2012.06.070.

Laminopathies: too much SUN is a bad thing

Daniel A Starr  1
Affiliations
Comment

Laminopathies: too much SUN is a bad thing

Daniel A Starr. Curr Biol. .

Abstract

SUN proteins accelerate the pathological progression of laminopathies. Although the mechanisms remain to be elucidated, an intriguing possibility is that high levels of SUN proteins lead to a hyperactive DNA damage response.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Models for how lamin A and SUN proteins contribute to pathologies of laminopathies. (A) A normal nucleus with lamin A (green), SUN (red), and KASH (blue) proteins at the nuclear envelope. (B) A deformed nucleus lacking lamin A with an over-accumulation of SUN proteins at the nuclear envelope. (C) The knockdown of SUN proteins rescues many of the pathologies of a lamin A mutant nucleus. See text for discussion of the four models.
See this image and copyright information in PMC

Comment on

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Worman HJ. Nuclear lamins and laminopathies. J Pathol. 2012;226:316–325. - PMC - PubMed
    1. Prokocimer M, Davidovich M, Nissim-Rafinia M, Wiesel-Motiuk N, Bar D, Barkan R, Meshorer E, Gruenbaum Y. Nuclear lamins: key regulators of nuclear structure and activities. J Cell Mol Med. 2009;13:1059–1085. - PMC - PubMed
    1. Chen CY, Chi YH, Mutalif RA, Starost MF, Myers TG, Anderson SA, Stewart CL, Jeang KT. Accumulation of the inner nuclear envelope protein sun1 is pathogenic in progeric and dystrophic laminopathies. Cell. 2012;149:565–577. - PMC - PubMed
    1. Lei K, Zhu X, Xu R, Shao C, Xu T, Zhuang Y, Han M. Inner nuclear envelope proteins SUN1 and SUN2 play a prominent role in the DNA damage response. Curr Biol. 2012;22:1609–1615. - PMC - PubMed
    1. Starr DA, Fridolfsson HN. Interactions between nuclei and the cytoskeleton are mediated by SUN-KASH nuclear-envelope bridges. Annu Rev Cell Dev Biol. 2010;26:421–444. - PMC - PubMed