Open Access

Frontiers in Genetics, volume 10

Are There Common Mechanisms Between the Hutchinson–Gilford Progeria Syndrome and Natural Aging?

Ashapkin Vasily V ¹

Kutueva Lyudmila I. ¹

Kurchashova Svetlana Y ¹

Kireev Igor I ¹

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Belozersky Research Institute of Physico-Chemical Biology, Lomonosov Moscow State University, Russia |

Publication type: Journal Article

Publication date: 2019-05-15

Frontiers Media S.A.

Journal: Frontiers in Genetics

Quartile SCImago

Quartile WOS

Impact factor: 3.7

ISSN: 16648021

DOI: 10.3389/FGENE.2019.00455

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PubMed ID: 31156709

Genetics

Molecular Medicine

Genetics(clinical)

Abstract

The Hutchinson–Gilford progeria syndrome (HGPS) is a premature aging disease caused by mutations of the LMNA gene leading to increased production of a partially processed form of the nuclear fibrillar protein lamin A – progerin. Progerin acts as a dominant factor that leads to multiple morphological anomalies of cell nuclei and disturbances in heterochromatin organization, mitosis, DNA replication and repair, and gene transcription. Progerin-positive cells are present in primary fibroblast cultures obtained from the skin of normal donors at advanced ages. These cells display HGPS-like defects in nuclear morphology, decreased H3K9me3 and HP1, and increased histone H2AX phosphorylation marks of the DNA damage loci. Inhibition of progerin production in cells of aged non-HGPS donors in vivo increases the proliferative activity, H3K9me3, and HP1, and decreases the senescence markers p21, IGFBP3, and GADD45B to the levels of young donor cells. Thus, progerin-dependent mechanisms act in natural aging. Excessive activity of the same mechanisms may well be the cause of premature aging in HGPS. Telomere attrition is widely regarded to be one of the primary hallmarks of aging. Progerin expression in normal human fibroblasts accelerates the loss of telomeres. Changes in lamina organization may directly affect telomere attrition resulting in accelerated replicative senescence and progeroid phenotypes. The chronological aging in normal individuals and the premature aging in HGPS patients are mediated by similar changes in the activity of signaling pathways, including downregulation of DNA repair and chromatin organization, and upregulation of ERK, mTOR, GH-IGF1, MAPK, TGFβ, and mitochondrial dysfunction. Multiple epigenetic changes are common to premature aging in HGPS and natural aging. Recent studies showed that epigenetic systems could play an active role as drivers of both forms of aging. It may be suggested that these systems translate the effects of various internal and external factors into universal molecular hallmarks, largely common between natural and accelerated forms of aging. Drugs acting at both natural aging and HGPS are likely to exist. For example, vitamin D3 reduces the progerin production and alleviates most HGPS features, and also slows down epigenetic aging in overweight and obese non-HGPS individuals with suboptimal vitamin D status.

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Citations by journals

	1 2 3 4 5 6
Cells	Cells, 6, 10.91% Cells 6 publications, 10.91%
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Genes	Genes, 2, 3.64% Genes 2 publications, 3.64%
International Journal of Molecular Sciences	International Journal of Molecular Sciences, 2, 3.64% International Journal of Molecular Sciences 2 publications, 3.64%
Scientific Reports	Scientific Reports, 2, 3.64% Scientific Reports 2 publications, 3.64%
DNA Repair	DNA Repair, 2, 3.64% DNA Repair 2 publications, 3.64%
Aging Cell	Aging Cell, 2, 3.64% Aging Cell 2 publications, 3.64%
Molecular Biology	Molecular Biology, 2, 3.64% Molecular Biology 2 publications, 3.64%
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Arteriosclerosis, Thrombosis, and Vascular Biology	Arteriosclerosis, Thrombosis, and Vascular Biology, 1, 1.82% Arteriosclerosis, Thrombosis, and Vascular Biology 1 publication, 1.82%
Marine Drugs	Marine Drugs, 1, 1.82% Marine Drugs 1 publication, 1.82%
International Journal of Environmental Research and Public Health	International Journal of Environmental Research and Public Health, 1, 1.82% International Journal of Environmental Research and Public Health 1 publication, 1.82%
Frontiers in Aging	Frontiers in Aging, 1, 1.82% Frontiers in Aging 1 publication, 1.82%
Hormones	Hormones, 1, 1.82% Hormones 1 publication, 1.82%
Clinical Epigenetics	Clinical Epigenetics, 1, 1.82% Clinical Epigenetics 1 publication, 1.82%
Nature Reviews Endocrinology	Nature Reviews Endocrinology, 1, 1.82% Nature Reviews Endocrinology 1 publication, 1.82%
Acta Histochemica	Acta Histochemica, 1, 1.82% Acta Histochemica 1 publication, 1.82%
Biophysical Journal	Biophysical Journal, 1, 1.82% Biophysical Journal 1 publication, 1.82%
Mutation Research - Fundamental and Molecular Mechanisms of Mutagenesis	Mutation Research - Fundamental and Molecular Mechanisms of Mutagenesis, 1, 1.82% Mutation Research - Fundamental and Molecular Mechanisms of Mutagenesis 1 publication, 1.82%
Biomaterials	Biomaterials, 1, 1.82% Biomaterials 1 publication, 1.82%
Journal of Neuroscience Research	Journal of Neuroscience Research, 1, 1.82% Journal of Neuroscience Research 1 publication, 1.82%
Wiley interdisciplinary reviews. RNA	Wiley interdisciplinary reviews. RNA, 1, 1.82% Wiley interdisciplinary reviews. RNA 1 publication, 1.82%
Carcinogenesis	Carcinogenesis, 1, 1.82% Carcinogenesis 1 publication, 1.82%
npj Aging	npj Aging, 1, 1.82% npj Aging 1 publication, 1.82%
Annual Review of Vision Science	Annual Review of Vision Science, 1, 1.82% Annual Review of Vision Science 1 publication, 1.82%
Langmuir	Langmuir, 1, 1.82% Langmuir 1 publication, 1.82%
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Citations by publishers

	2 4 6 8 10 12
Multidisciplinary Digital Publishing Institute (MDPI)	Multidisciplinary Digital Publishing Institute (MDPI), 12, 21.82% Multidisciplinary Digital Publishing Institute (MDPI) 12 publications, 21.82%
Springer Nature	Springer Nature, 7, 12.73% Springer Nature 7 publications, 12.73%
Elsevier	Elsevier, 7, 12.73% Elsevier 7 publications, 12.73%
Wiley	Wiley, 4, 7.27% Wiley 4 publications, 7.27%
Bentham Science	Bentham Science, 2, 3.64% Bentham Science 2 publications, 3.64%
Impact Journals	Impact Journals, 2, 3.64% Impact Journals 2 publications, 3.64%
Pleiades Publishing	Pleiades Publishing, 2, 3.64% Pleiades Publishing 2 publications, 3.64%
The Endocrine Society	The Endocrine Society, 1, 1.82% The Endocrine Society 1 publication, 1.82%
IOS Press	IOS Press, 1, 1.82% IOS Press 1 publication, 1.82%
Wolters Kluwer Health	Wolters Kluwer Health, 1, 1.82% Wolters Kluwer Health 1 publication, 1.82%
Frontiers Media S.A.	Frontiers Media S.A., 1, 1.82% Frontiers Media S.A. 1 publication, 1.82%
Biophysical Society	Biophysical Society, 1, 1.82% Biophysical Society 1 publication, 1.82%
Oxford University Press	Oxford University Press, 1, 1.82% Oxford University Press 1 publication, 1.82%
Annual Reviews	Annual Reviews, 1, 1.82% Annual Reviews 1 publication, 1.82%
American Chemical Society (ACS)	American Chemical Society (ACS), 1, 1.82% American Chemical Society (ACS) 1 publication, 1.82%
Bioscientifica	Bioscientifica, 1, 1.82% Bioscientifica 1 publication, 1.82%
Taylor & Francis	Taylor & Francis, 1, 1.82% Taylor & Francis 1 publication, 1.82%
	2 4 6 8 10 12

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Ashapkin V. V. et al. Are There Common Mechanisms Between the Hutchinson–Gilford Progeria Syndrome and Natural Aging? // Frontiers in Genetics. 2019. Vol. 10.

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Ashapkin V. V., Kutueva L. I., Kurchashova S. Y., Kireev I. I. Are There Common Mechanisms Between the Hutchinson–Gilford Progeria Syndrome and Natural Aging? // Frontiers in Genetics. 2019. Vol. 10.

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