B-type lamins in health and disease - PubMed (original) (raw)

Review

B-type lamins in health and disease

C J Hutchison. Semin Cell Dev Biol. 2014 May.

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.

Keywords: Autosomal dominant leukodystrophy; Brain development; Cellular senescence; Lamin B1.

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Figures

Fig. 1

The effects of different cellular stresses that lead to senescence on lamin A to lamin B1 ratios. Healthy early passage cells have a balanced LMA to LB1 ratio. In response to senescence-inducing events, LB1 expression is altered and/or LMA is oxidized. This figure was reproduced with kind permission from EMBO Journal.

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B-type lamins in health and disease - PubMed (original) (raw)