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The nuclear lamina in health and disease

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Agnieszka Dobrzynska, Susana Gonzalo, Cathy Shanahan, Peter Askjaer

Original languageEnglish
Pages (from-to)233-248
Number of pages16
JournalNucleus-Austin
Volume7
Issue number3
Early online date9 May 2016
DOIs
Publication statusE-pub ahead of print - 9 May 2016

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Abstract

The nuclear lamina (NL) is a structural component of the nuclear envelope and makes extensive contacts with integral nuclear membrane proteins and chromatin. These interactions are critical for many cellular processes, such as nuclear positioning, perception of mechanical stimuli from the cell surface, nuclear stability, 3-dimensional organization of chromatin and regulation of chromatin-binding proteins, including transcription factors. The NL is present in all nucleated metazoan cells but its composition and interactome differ between tissues. Most likely, this contributes to the broad spectrum of disease manifestations in humans with mutations in NL-related genes, ranging from muscle dystrophies to neurological disorders, lipodystrophies and progeria syndromes. We review here exciting novel insight into NL function at the cellular level, in particular in chromatin organization and mechanosensation. We also present recent observations on the relation between the NL and metabolism and the special relevance of the NL in muscle tissues. Finally, we discuss new therapeutic approaches to treat NL-related diseases.

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