The Nuclear Lamina: Protein Accumulation and Disease

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 e...

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Main Authors:	Carla Almendáriz-Palacios, Zoe E. Gillespie, Matthew Janzen, Valeria Martinez, Joanna M. Bridger, Troy A. A. Harkness, Darrell D. Mousseau, Christopher H. Eskiw
Format:	Article
Language:	English
Published:	MDPI AG 2020-07-01
Series:	Biomedicines
Subjects:	lamina protein accumulation premature aging neurodegeneration autophagy clearance
Online Access:	https://www.mdpi.com/2227-9059/8/7/188

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spelling	doaj-dabe5c2be942465da22e52566196e34a2020-11-25T02:22:15ZengMDPI AGBiomedicines2227-90592020-07-01818818810.3390/biomedicines8070188The Nuclear Lamina: Protein Accumulation and DiseaseCarla Almendáriz-Palacios0Zoe E. Gillespie1Matthew Janzen2Valeria Martinez3Joanna M. Bridger4Troy A. A. Harkness5Darrell D. Mousseau6Christopher H. Eskiw7Department of Food and Bioproduct Sciences, University of Saskatchewan, Saskatoon, SK S7N 5A8, CanadaDepartment of Biochemistry, Microbiology and Immunology, University of Saskatchewan, Saskatoon, SK S7N 5A8, CanadaDepartment of Biochemistry, Microbiology and Immunology, University of Saskatchewan, Saskatoon, SK S7N 5A8, CanadaDepartment of Food and Bioproduct Sciences, University of Saskatchewan, Saskatoon, SK S7N 5A8, CanadaCentre for Genome Engineering and Maintenance, College of Health, Life and Medical Sciences, Brunel University London, Kingston Lane, Uxbridge UB8 3PH, UKDepartment of Biochemistry, Microbiology and Immunology, University of Saskatchewan, Saskatoon, SK S7N 5A8, CanadaCell Signalling Laboratory, Department of Psychiatry, University of Saskatchewan, Saskatoon, SK S7N 5A5, CanadaDepartment of Food and Bioproduct Sciences, University of Saskatchewan, Saskatoon, SK S7N 5A8, CanadaCellular 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.https://www.mdpi.com/2227-9059/8/7/188laminaprotein accumulationpremature agingneurodegenerationautophagyclearance
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Carla Almendáriz-Palacios Zoe E. Gillespie Matthew Janzen Valeria Martinez Joanna M. Bridger Troy A. A. Harkness Darrell D. Mousseau Christopher H. Eskiw
spellingShingle	Carla Almendáriz-Palacios Zoe E. Gillespie Matthew Janzen Valeria Martinez Joanna M. Bridger Troy A. A. Harkness Darrell D. Mousseau Christopher H. Eskiw The Nuclear Lamina: Protein Accumulation and Disease Biomedicines lamina protein accumulation premature aging neurodegeneration autophagy clearance
author_facet	Carla Almendáriz-Palacios Zoe E. Gillespie Matthew Janzen Valeria Martinez Joanna M. Bridger Troy A. A. Harkness Darrell D. Mousseau Christopher H. Eskiw
author_sort	Carla Almendáriz-Palacios
title	The Nuclear Lamina: Protein Accumulation and Disease
title_short	The Nuclear Lamina: Protein Accumulation and Disease
title_full	The Nuclear Lamina: Protein Accumulation and Disease
title_fullStr	The Nuclear Lamina: Protein Accumulation and Disease
title_full_unstemmed	The Nuclear Lamina: Protein Accumulation and Disease
title_sort	nuclear lamina: protein accumulation and disease
publisher	MDPI AG
series	Biomedicines
issn	2227-9059
publishDate	2020-07-01
description	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.
topic	lamina protein accumulation premature aging neurodegeneration autophagy clearance
url	https://www.mdpi.com/2227-9059/8/7/188
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The Nuclear Lamina: Protein Accumulation and Disease

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