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The Nuclear Lamina: Protein Accumulation and Disease.

ABSTRACT: 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.

SUBMITTER: Almendariz-Palacios C

PROVIDER: S-EPMC7400325 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature

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The Nuclear Lamina: Protein Accumulation and Disease.

Almendáriz-Palacios Carla C Gillespie Zoe E ZE Janzen Matthew M Martinez Valeria V Bridger Joanna M JM Harkness Troy A A TAA Mousseau Darrell D DD Eskiw Christopher H CH

Biomedicines 20200701 7

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 resul ...[more]

PMID: 32630170

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