B-type lamins in health and disease

Hutchison, C.J.

doi:10.1016/j.semcdb.2013.12.012

B-type lamins in health and disease

Hutchison, C.J.

Authors

C.J. Hutchison

Abstract

For over two decades, B-type lamins were thought to have roles in fundamental processes including correct assembly of nuclear envelopes, DNA replication, transcription and cell survival. Recent studies have questioned these roles and have instead emphasised the role of these proteins in tissue building and tissue integrity, particularly in tissues devoid of A-type lamins. Other studies have suggested that the expression of B-type lamins in somatic cells influences the rate of entry into states of cellular senescence. In humans duplication of the LMNB1 gene (encoding lamin B1) causes an adult onset neurodegenerative disorder, termed autosomal dominant leukodystrophy, whilst very recently, LMNB1 has been implicated as a susceptibility gene in neural tube defects. This is consistent with studies in mice that reveal a critical role for B-type lamins in neuronal migration and brain development. In this review, I will consider how different model systems have contributed to our understanding of the functions of B-type lamins and which of those functions are critical for human health and disease.

Citation

Hutchison, C. (2014). B-type lamins in health and disease. Seminars in Cell and Developmental Biology, 29, 158-163. https://doi.org/10.1016/j.semcdb.2013.12.012

Journal Article Type	Article
Publication Date	May 1, 2014
Deposit Date	Jan 10, 2014
Publicly Available Date	Mar 25, 2014
Journal	Seminars in Cell and Developmental Biology
Print ISSN	1084-9521
Publisher	Elsevier
Peer Reviewed	Peer Reviewed
Volume	29
Pages	158-163
DOI	https://doi.org/10.1016/j.semcdb.2013.12.012
Keywords	Lamin B1, Cellular senescence, Brain development, Autosomal dominant leukodystrophy.

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