Soft substrates normalize nuclear morphology and prevent nuclear rupture in fibroblasts from a laminopathy patient with compound heterozygous LMNA mutations

Laminopathies, mainly caused by mutations in the LMNA gene, are a group of inherited diseases with a highly variable penetrance; i.e., the disease spectrum in persons with identical LMNA mutations range from symptom-free conditions to severe cardiomyopathy and progeria, leading to early death. LMNA...

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Main Authors: Tamiello, Chiara, Kamps, Miriam A.F., van den Wijngaard, Arthur, Verstraeten, Valerie L. R. M., Baaijens, Frank P.T., Broers, Jos L.V., Bouten, Carlijn C.V.
Formato: Artigo
Idioma:Inglês
Publicado em: Landes Bioscience 2013
Assuntos:
Research Paper
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3585029/
https://ncbi.nlm.nih.gov/pubmed/23324461
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.4161/nucl.23388
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