- Title
- B-type lamins in health and disease
- Creator
- Hutchison, Chris
- Date
- 2014
- Type
- Text; Journal article
- Identifier
- http://researchonline.federation.edu.au/vital/access/HandleResolver/1959.17/170808
- Identifier
- vital:14210
- Identifier
-
https://doi.org/10.1016/j.semcdb.2013.12.012
- Identifier
- ISBN:1084-9521
- 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. © 2014 The Author.
- Publisher
- Elsevier Ltd
- Relation
- Seminars in Cell and Developmental Biology Vol. 29, no. (2014), p. 158-163
- Rights
- https://creativecommons.org/licenses/by/3.0/
- Rights
- Open Access
- Rights
- This metadata is freely available under a CCO license
- Subject
- Autosomal dominant leukodystrophy; Brain development; Cellular senescence; Lamin B1; DNA; Lamin B; Messenger RNA; Myelin basic protein; Proteolipid protein; Unclassified drug; Cell aging; Cell cycle arrest; Cell nucleus membrane; Cell proliferation; Cell structure; Chromosome segregation; Chromosome structure; Degenerative disease; Disease association; DNA end joining repair; DNA replication; DNA transcription; Down regulation; Gene activation; Gene silencing; Genetic susceptibility; Human; Leukodystrophy; Mitosis spindle; Neural tube defect; Nuclear lamina; Nuclear pore complex; Oxidative stress; Protein function; Spindle pole; Transcription regulation; Animal; Cell motion; Cytology; Gene duplication; Genetic transcription; Genetics; Metabolism; Mouse; Nerve cell; Spindle apparatus; Mus; Animals; Cell Movement; Humans; Lamin Type B; Mice; Neural Tube Defects; Neurodegenerative Diseases; Neurons; Transcription, Genetic; 0601 Biochemistry and Cell Biology; 1114 Paediatrics and Reproductive Medicine
- Full Text
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