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Review
. 2011 Mar;16(1):114-48.
doi: 10.2478/s11658-010-0038-9. Epub 2010 Dec 27.

Laminopathies: the molecular background of the disease and the prospects for its treatment

Magdalena Zaremba-Czogalla  1 , Magda Dubińska-Magiera, Ryszard Rzepecki
Affiliations
Review

Laminopathies: the molecular background of the disease and the prospects for its treatment

Magdalena Zaremba-Czogalla et al. Cell Mol Biol Lett. 2011 Mar.

Abstract

Laminopathies are rare human degenerative disorders with a wide spectrum of clinical phenotypes, associated with defects in the main protein components of the nuclear envelope, mostly in the lamins. They include systemic disorders and tissue-restricted diseases. Scientists have been trying to explain the pathogenesis of laminopathies and find an efficient method for treatment for many years. In this review, we discuss the current state of knowledge about laminopathies, the molecular mechanisms behind the development of particular phenotypes, and the prospects for stem cell and/or gene therapy treatments.

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