Abstract |
Much of the work on nuclear lamins during the past 15 years has focused on mutations in LMNA (the gene for prelamin A and lamin C) that cause particular muscular dystrophy, cardiomyopathy, partial lipodystrophy, and progeroid syndromes. These disorders, often called " laminopathies," mainly affect mesenchymal tissues (e.g., striated muscle, bone, and fibrous tissue). Recently, however, a series of papers have identified important roles for nuclear lamins in the central nervous system. Studies of knockout mice uncovered a key role for B-type lamins ( lamins B1 and B2) in neuronal migration in the developing brain. Also, duplications of LMNB1 (the gene for lamin B1) have been shown to cause autosome-dominant leukodystrophy. Finally, recent studies have uncovered a peculiar pattern of nuclear lamin expression in the brain. Lamin C transcripts are present at high levels in the brain, but prelamin A expression levels are very low-due to regulation of prelamin A transcripts by microRNA 9. This form of prelamin A regulation likely explains why " prelamin A diseases" such as Hutchinson-Gilford progeria syndrome spare the central nervous system. In this review, we summarize recent progress in elucidating links between nuclear lamins and neurobiology.
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Authors | Stephen G Young, Hea-Jin Jung, John M Lee, Loren G Fong |
Journal | Molecular and cellular biology
(Mol Cell Biol)
Vol. 34
Issue 15
Pg. 2776-85
(Aug 2014)
ISSN: 1098-5549 [Electronic] United States |
PMID | 24842906
(Publication Type: Journal Article, Research Support, N.I.H., Extramural, Review)
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Copyright | Copyright © 2014, American Society for Microbiology. All Rights Reserved. |
Chemical References |
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Topics |
- Animals
- Humans
- Lamins
(genetics, metabolism)
- Neurobiology
(methods)
- Nuclear Lamina
(genetics, metabolism)
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