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The Nuclear Lamina: Protein Accumulation and Disease

1
Department of Food and Bioproduct Sciences, University of Saskatchewan, Saskatoon, SK S7N 5A8, Canada
2
Department of Biochemistry, Microbiology and Immunology, University of Saskatchewan, Saskatoon, SK S7N 5A8, Canada
3
Centre for Genome Engineering and Maintenance, College of Health, Life and Medical Sciences, Brunel University London, Kingston Lane, Uxbridge UB8 3PH, UK
4
Cell Signalling Laboratory, Department of Psychiatry, University of Saskatchewan, Saskatoon, SK S7N 5A5, Canada
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Biomedicines 2020, 8(7), 188; https://doi.org/10.3390/biomedicines8070188
Received: 1 June 2020 / Revised: 23 June 2020 / Accepted: 26 June 2020 / Published: 1 July 2020
(This article belongs to the Special Issue Protein Structure, Function and Dynamics in Diseases and Therapeutics)
Cellular health is reliant on proteostasis—the maintenance of protein levels regulated through multiple pathways modulating protein synthesis, degradation and clearance. Loss of proteostasis results in serious disease and is associated with aging. One proteinaceous structure underlying the nuclear envelope—the nuclear lamina—coordinates essential processes including DNA repair, genome organization and epigenetic and transcriptional regulation. Loss of proteostasis within the nuclear lamina results in the accumulation of proteins, disrupting these essential functions, either via direct interactions of protein aggregates within the lamina or by altering systems that maintain lamina structure. Here we discuss the links between proteostasis and disease of the nuclear lamina, as well as how manipulating specific proteostatic pathways involved in protein clearance could improve cellular health and prevent/reverse disease. View Full-Text
Keywords: lamina; protein accumulation; premature aging; neurodegeneration; autophagy; clearance lamina; protein accumulation; premature aging; neurodegeneration; autophagy; clearance
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Almendáriz-Palacios, C.; Gillespie, Z.E.; Janzen, M.; Martinez, V.; Bridger, J.M.; Harkness, T.A.A.; Mousseau, D.D.; Eskiw, C.H. The Nuclear Lamina: Protein Accumulation and Disease. Biomedicines 2020, 8, 188.

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