Aberrant Function of the C-Terminal Tail of HIST1H1E Accelerates Cellular Senescence and Causes Premature Aging.

HIST1H1E accelerated aging cellular senescence chromatin compaction chromatin dynamics chromatin remodeling linker histone linker histone H1.4 methylation profiling replicative senescence

Journal

American journal of human genetics
ISSN: 1537-6605
Titre abrégé: Am J Hum Genet
Pays: United States
ID NLM: 0370475

Informations de publication

Date de publication:
05 09 2019
Historique:
received: 06 04 2019
accepted: 10 07 2019
pmc-release: 05 03 2020
pubmed: 27 8 2019
medline: 3 4 2020
entrez: 27 8 2019
Statut: ppublish

Résumé

Histones mediate dynamic packaging of nuclear DNA in chromatin, a process that is precisely controlled to guarantee efficient compaction of the genome and proper chromosomal segregation during cell division and to accomplish DNA replication, transcription, and repair. Due to the important structural and regulatory roles played by histones, it is not surprising that histone functional dysregulation or aberrant levels of histones can have severe consequences for multiple cellular processes and ultimately might affect development or contribute to cell transformation. Recently, germline frameshift mutations involving the C-terminal tail of HIST1H1E, which is a widely expressed member of the linker histone family and facilitates higher-order chromatin folding, have been causally linked to an as-yet poorly defined syndrome that includes intellectual disability. We report that these mutations result in stable proteins that reside in the nucleus, bind to chromatin, disrupt proper compaction of DNA, and are associated with a specific methylation pattern. Cells expressing these mutant proteins have a dramatically reduced proliferation rate and competence, hardly enter into the S phase, and undergo accelerated senescence. Remarkably, clinical assessment of a relatively large cohort of subjects sharing these mutations revealed a premature aging phenotype as a previously unrecognized feature of the disorder. Our findings identify a direct link between aberrant chromatin remodeling, cellular senescence, and accelerated aging.

Identifiants

pubmed: 31447100
pii: S0002-9297(19)30270-8
doi: 10.1016/j.ajhg.2019.07.007
pmc: PMC6731364
pii:
doi:

Substances chimiques

Chromatin 0
Histones 0

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

493-508

Subventions

Organisme : NIGMS NIH HHS
ID : T32 GM007748
Pays : United States
Organisme : NHGRI NIH HHS
ID : UM1 HG008900
Pays : United States

Informations de copyright

Copyright © 2019 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

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Auteurs

Elisabetta Flex (E)

Department of Oncology and Molecular Medicine, Istituto Superiore di Sanità, Rome, 00161 Italy; Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY 10467, USA.

Simone Martinelli (S)

Department of Oncology and Molecular Medicine, Istituto Superiore di Sanità, Rome, 00161 Italy.

Anke Van Dijck (A)

Department of Medical Genetics, University of Antwerp, Edegem, 2650 Belgium; Department of Neurology, Antwerp University Hospital, Edegem, 2650 Belgium.

Andrea Ciolfi (A)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy.

Serena Cecchetti (S)

Microscopy Area, Core Facilities, Istituto Superiore di Sanità, Rome, 00161 Italy.

Elisa Coluzzi (E)

Department of Science, University Roma Tre, Rome, 00146 Italy.

Luca Pannone (L)

Department of Oncology and Molecular Medicine, Istituto Superiore di Sanità, Rome, 00161 Italy; Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy.

Cristina Andreoli (C)

Department of Environment and Health, Istituto Superiore di Sanità, Rome, 00161 Italy.

Francesca Clementina Radio (FC)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy.

Simone Pizzi (S)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy.

Giovanna Carpentieri (G)

Department of Oncology and Molecular Medicine, Istituto Superiore di Sanità, Rome, 00161 Italy; Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy.

Alessandro Bruselles (A)

Department of Oncology and Molecular Medicine, Istituto Superiore di Sanità, Rome, 00161 Italy.

Giuseppina Catanzaro (G)

Department of Experimental Medicine, Sapienza University, Rome, 00161 Italy.

Lucia Pedace (L)

Department of Pediatric Onco-Hematology and Cell and Gene Therapy, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, 00146 Italy.

Evelina Miele (E)

Department of Pediatric Onco-Hematology and Cell and Gene Therapy, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, 00146 Italy.

Elena Carcarino (E)

Department of Pediatric Onco-Hematology and Cell and Gene Therapy, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, 00146 Italy; Current affiliation: Cordeliers Research Centre, Inserm 1138, Sorbonne Université, Paris, 75006 France.

Xiaoyan Ge (X)

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA; Current affiliation: Department of Genetics and Genomic Sciences, The Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.

Chieko Chijiwa (C)

Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V6H 3N1, Canada.

M E Suzanne Lewis (MES)

Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V6H 3N1, Canada.

Marije Meuwissen (M)

Department of Medical Genetics, University of Antwerp, Edegem, 2650 Belgium.

Sandra Kenis (S)

Department of Neurology, Antwerp University Hospital, Edegem, 2650 Belgium.

Nathalie Van der Aa (N)

Department of Medical Genetics, University of Antwerp, Edegem, 2650 Belgium.

Austin Larson (A)

Section of Clinical Genetics and Metabolism, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO 80045, USA.

Kathleen Brown (K)

Section of Clinical Genetics and Metabolism, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO 80045, USA.

Melissa P Wasserstein (MP)

Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY 10467, USA.

Brian G Skotko (BG)

Division of Medical Genetics and Metabolism, Department of Pediatrics, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; Department of Pediatrics, Harvard Medical School, Boston, MA 02114, USA.

Amber Begtrup (A)

GeneDx, Gaithersburg, MD 20877, USA.

Richard Person (R)

GeneDx, Gaithersburg, MD 20877, USA.

Maria Karayiorgou (M)

Department of Psychiatry, Columbia University Medical Center, New York, NY 10032, USA.

J Louw Roos (JL)

Department of Psychiatry, University of Pretoria, Weskoppies Hospital, Pretoria, 0001 South Africa.

Koen L Van Gassen (KL)

Department of Genetics, Center for Molecular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, 3508 AB the Netherlands.

Marije Koopmans (M)

Department of Genetics, Center for Molecular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, 3508 AB the Netherlands.

Emilia K Bijlsma (EK)

Department of Clinical Genetics, Leiden University Medical Center, Leiden, 2300 RC the Netherlands.

Gijs W E Santen (GWE)

Department of Clinical Genetics, Leiden University Medical Center, Leiden, 2300 RC the Netherlands.

Daniela Q C M Barge-Schaapveld (DQCM)

Department of Clinical Genetics, Leiden University Medical Center, Leiden, 2300 RC the Netherlands.

Claudia A L Ruivenkamp (CAL)

Department of Clinical Genetics, Leiden University Medical Center, Leiden, 2300 RC the Netherlands.

Mariette J V Hoffer (MJV)

Department of Clinical Genetics, Leiden University Medical Center, Leiden, 2300 RC the Netherlands.

Seema R Lalani (SR)

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

Haley Streff (H)

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

William J Craigen (WJ)

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

Brett H Graham (BH)

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA; Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

Annette P M van den Elzen (APM)

Departement of Pediatrics, Reinier de Graaf Ziekenhuis, Delft, 2600 GA the Netherlands.

Daan J Kamphuis (DJ)

Departement of Neurology, Reinier de Graaf Ziekenhuis, Delft, 2600 GA the Netherlands.

Katrin Õunap (K)

Department of Clinical Genetics, United Laboratories, Tartu University Hospital, Tartu, 50406 Estonia; Institute of Clinical Medicine, University of Tartu, Tartu, 50406 Estonia.

Karit Reinson (K)

Department of Clinical Genetics, United Laboratories, Tartu University Hospital, Tartu, 50406 Estonia; Institute of Clinical Medicine, University of Tartu, Tartu, 50406 Estonia.

Sander Pajusalu (S)

Department of Clinical Genetics, United Laboratories, Tartu University Hospital, Tartu, 50406 Estonia; Institute of Clinical Medicine, University of Tartu, Tartu, 50406 Estonia; Department of Genetics, Yale University School of Medicine, New Haven, CT 06510, USA.

Monica H Wojcik (MH)

Broad Institute of MIT and Harvard, Cambridge, MA 02142, USA.

Clara Viberti (C)

Department of Medical Sciences, University of Turin, Turin, 10126 Italy; Italian Institute for Genomic Medicine, Turin, 10126 Italy.

Cornelia Di Gaetano (C)

Department of Medical Sciences, University of Turin, Turin, 10126 Italy; Italian Institute for Genomic Medicine, Turin, 10126 Italy.

Enrico Bertini (E)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy.

Simona Petrucci (S)

Department of Clinical and Molecular Medicine, Sapienza University, Rome, 00189 Italy; Division of Medical Genetics, Casa Sollievo della Sofferenza Hospital, IRCCS, San Giovanni Rotondo, 71013 Italy.

Alessandro De Luca (A)

Division of Medical Genetics, Casa Sollievo della Sofferenza Hospital, IRCCS, San Giovanni Rotondo, 71013 Italy.

Rossella Rota (R)

Department of Pediatric Onco-Hematology and Cell and Gene Therapy, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, 00146 Italy.

Elisabetta Ferretti (E)

Department of Experimental Medicine, Sapienza University, Rome, 00161 Italy; Istituto Neuromed, IRCCS, Pozzilli, 86077 Italy.

Giuseppe Matullo (G)

Department of Medical Sciences, University of Turin, Turin, 10126 Italy; Italian Institute for Genomic Medicine, Turin, 10126 Italy.

Bruno Dallapiccola (B)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy.

Antonella Sgura (A)

Department of Science, University Roma Tre, Rome, 00146 Italy.

Magdalena Walkiewicz (M)

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA; Current affiliation: National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, MD 20892, USA.

R Frank Kooy (RF)

Department of Medical Genetics, University of Antwerp, Edegem, 2650 Belgium. Electronic address: frank.kooy@uantwerpen.be.

Marco Tartaglia (M)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, 00146 Italy. Electronic address: marco.tartaglia@opbg.net.

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Classifications MeSH