Lamins and Lamin-Associated Proteins in Gastrointestinal Health and Disease.

Autor:	Brady GF; Department of Molecular and Integrative Physiology, University of Michigan Medical School, Ann Arbor, Michigan; Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan. Electronic address: gfbrady@med.umich.edu., Kwan R; Department of Molecular and Integrative Physiology, University of Michigan Medical School, Ann Arbor, Michigan., Bragazzi Cunha J; Department of Molecular and Integrative Physiology, University of Michigan Medical School, Ann Arbor, Michigan., Elenbaas JS; Medical Scientist Training Program, Washington University, St Louis, Missouri., Omary MB; Department of Molecular and Integrative Physiology, University of Michigan Medical School, Ann Arbor, Michigan; Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan; Åbo Akademi University, Turku, Finland.
Jazyk:	angličtina
Zdroj:	Gastroenterology [Gastroenterology] 2018 May; Vol. 154 (6), pp. 1602-1619.e1. Date of Electronic Publication: 2018 Mar 13.
DOI:	10.1053/j.gastro.2018.03.026
Abstrakt:	The nuclear lamina is a multi-protein lattice composed of A- and B-type lamins and their associated proteins. This protein lattice associates with heterochromatin and integral inner nuclear membrane proteins, providing links among the genome, nucleoskeleton, and cytoskeleton. In the 1990s, mutations in EMD and LMNA were linked to Emery-Dreifuss muscular dystrophy. Since then, the number of diseases attributed to nuclear lamina defects, including laminopathies and other disorders, has increased to include more than 20 distinct genetic syndromes. Studies of patients and mouse genetic models have pointed to important roles for lamins and their associated proteins in the function of gastrointestinal organs, including liver and pancreas. We review the interactions and functions of the lamina in relation to the nuclear envelope and genome, the ways in which its dysfunction is thought to contribute to human disease, and possible avenues for targeted therapies. (Copyright © 2018 AGA Institute. Published by Elsevier Inc. All rights reserved.)
Databáze:	MEDLINE
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