From loss to gain: role for SUN1 in laminopathies

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 concomit...

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Detalles Bibliográficos
Autores principales: Liu, Baohua, Jin, Dong-Yan, Zhou, Zhongjun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2012
Materias:
Research Highlight
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3419603/
https://www.ncbi.nlm.nih.gov/pubmed/22709970
http://dx.doi.org/10.1186/2045-3701-2-21
Descripción
Sumario: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.

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