Dialing Down SUN1 for Laminopathies
نویسندگان
چکیده
Laminopathies, caused by mutations in A-type nuclear lamins, encompass a range of diseases, including forms of progeria and muscular dystrophy. In this issue, Chen et al. provide evidence that elevated expression of the nuclear inner membrane protein SUN1 drives pathology in multiple laminopathies.
منابع مشابه
Accumulation of the Inner Nuclear Envelope Protein Sun1 Is Pathogenic in Progeric and Dystrophic Laminopathies
Human LMNA gene mutations result in laminopathies that include Emery-Dreifuss muscular dystrophy (AD-EDMD) and Hutchinson-Gilford progeria, the premature aging syndrome (HGPS). The Lmna null (Lmna(-/-)) and progeroid LmnaΔ9 mutant mice are models for AD-EDMD and HGPS, respectively. Both animals develop severe tissue pathologies with abbreviated life spans. Like HGPS cells, Lmna(-/-) and LmnaΔ9 ...
متن کاملDysregulated interactions between lamin A and SUN1 induce abnormalities in the nuclear envelope and endoplasmic reticulum in progeric laminopathies.
Hutchinson-Gilford progeria syndrome (HGPS) is a human progeroid disease caused by a point mutation on the LMNA gene. We reported previously that the accumulation of the nuclear envelope protein SUN1 contributes to HGPS nuclear aberrancies. However, the mechanism by which interactions between mutant lamin A (also known as progerin or LAΔ50) and SUN1 produce HGPS cellular phenotypes requires fur...
متن کاملThe Study of SAD1/UNC-84 Domain Protein 1 in Muscle of Diabetic Male Wistar Rats
Objective: The purpose of this study was to measure the changes of the SUN1 protein levels on soleus muscle in diabetic male wistar rats. Materials and Methods: Twenty male Wistar rats with 10 weeks old and weighing 200-250 grams were selected. After two weeks, the rats were divided into two groups (diabetic group and healthy group). After 12 hours fasting, diabetes was induced by intraperiton...
متن کاملMammalian SUN Protein Interaction Networks at the Inner Nuclear Membrane and Their Role in Laminopathy Disease Processes*
The nuclear envelope (NE) LINC complex, in mammals comprised of SUN domain and nesprin proteins, provides a direct connection between the nuclear lamina and the cytoskeleton, which contributes to nuclear positioning and cellular rigidity. SUN1 and SUN2 interact with lamin A, but lamin A is only required for NE localization of SUN2, and it remains unclear how SUN1 is anchored. Here, we identify ...
متن کاملDialing Torque Preferences of People with Diabetes When Using Insulin Pens: A Pilot Study
INTRODUCTION Ergonomic dialing torque may enhance safety and comfort when setting doses with insulin pens. Limited data are available on the correlation of dialing torque and dialing comfort. METHODS Three studies were performed with SoloSTAR(®) (SS; Sanofi), FlexPen(®) (FP; Novo Nordisk), KwikPen(®) (KP; Eli Lilly) and FlexTouch(®) (FT; Novo Nordisk) pens. Dialing behavior was examined with ...
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عنوان ژورنال:
- Cell
دوره 149 شماره
صفحات -
تاریخ انتشار 2012