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Nuclear Lamins in the Brain - New Insights into Function and Regulation

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Journal Mol Neurobiol
Date 2012 Oct 16
PMID 23065386
Citations 21
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Abstract

The nuclear lamina is an intermediate filament meshwork composed largely of four nuclear lamins - lamins A and C (A-type lamins) and lamins B1 and B2 (B-type lamins). Located immediately adjacent to the inner nuclear membrane, the nuclear lamina provides a structural scaffolding for the cell nucleus. It also interacts with both nuclear membrane proteins and the chromatin and is thought to participate in many important functions within the cell nucleus. Defects in A-type lamins cause cardiomyopathy, muscular dystrophy, peripheral neuropathy, lipodystrophy, and progeroid disorders. In contrast, the only bona fide link between the B-type lamins and human disease is a rare demyelinating disease of the central nervous system - adult-onset autosomal-dominant leukoencephalopathy, caused by a duplication of the gene for lamin B1. However, this leukoencephalopathy is not the only association between the brain and B-type nuclear lamins. Studies of conventional and tissue-specific knockout mice have demonstrated that B-type lamins play essential roles in neuronal migration in the developing brain and in neuronal survival. The importance of A-type lamin expression in the brain is unclear, but it is intriguing that the adult brain preferentially expresses lamin C rather than lamin A, very likely due to microRNA-mediated removal of prelamin A transcripts. Here, we review recent studies on nuclear lamins, focusing on the function and regulation of the nuclear lamins in the central nervous system.

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References
1.
Rober R, Weber K, Osborn M . Differential timing of nuclear lamin A/C expression in the various organs of the mouse embryo and the young animal: a developmental study. Development. 1989; 105(2):365-78. DOI: 10.1242/dev.105.2.365. View

2.
Nissan X, Blondel S, Navarro C, Maury Y, Denis C, Girard M . Unique preservation of neural cells in Hutchinson- Gilford progeria syndrome is due to the expression of the neural-specific miR-9 microRNA. Cell Rep. 2012; 2(1):1-9. DOI: 10.1016/j.celrep.2012.05.015. View

3.
Broers J, Ramaekers F, Bonne G, Ben Yaou R, Hutchison C . Nuclear lamins: laminopathies and their role in premature ageing. Physiol Rev. 2006; 86(3):967-1008. DOI: 10.1152/physrev.00047.2005. View

4.
Brussino A, Vaula G, Cagnoli C, Panza E, Seri M, Di Gregorio E . A family with autosomal dominant leukodystrophy linked to 5q23.2-q23.3 without lamin B1 mutations. Eur J Neurol. 2009; 17(4):541-9. DOI: 10.1111/j.1468-1331.2009.02844.x. View

5.
Gao F . Context-dependent functions of specific microRNAs in neuronal development. Neural Dev. 2010; 5:25. PMC: 2958854. DOI: 10.1186/1749-8104-5-25. View