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Title

The nuclear lamina in health and disease

AuthorsDobrzynska, Agnieszka ; Gonzalo, Susana; Shanahan, Catherine; Askjaer, Peter
KeywordsFarnesylation
Metabolism
Muclear lamina
Muclear envelope
Chromatin
Emerin
Muscles
Hutchinson-Gilford progeria syndrome
Laminopathies
LAP2
LBR
Mechanotransduction
Issue Date2016
PublisherTaylor & Francis
CitationNucleus 7(3): 233-248 (2016)
AbstractThe 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.
Publisher version (URL)http://dx.doi.org/10.1080/19491034.2016.1183848
URIhttp://hdl.handle.net/10261/163425
Identifiersdoi: 10.1080/19491034.2016.1183848
issn: 1949-1042
Appears in Collections:(CABD) Artículos
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