Frameshift mutations at the C-terminus of HIST1H1E result in a specific DNA hypomethylation signature.


Journal

Clinical epigenetics
ISSN: 1868-7083
Titre abrégé: Clin Epigenetics
Pays: Germany
ID NLM: 101516977

Informations de publication

Date de publication:
07 01 2020
Historique:
received: 11 09 2019
accepted: 26 12 2019
entrez: 9 1 2020
pubmed: 9 1 2020
medline: 17 8 2021
Statut: epublish

Résumé

We previously associated HIST1H1E mutations causing Rahman syndrome with a specific genome-wide methylation pattern. Methylome analysis from peripheral blood samples of six affected subjects led us to identify a specific hypomethylated profile. This "episignature" was enriched for genes involved in neuronal system development and function. A computational classifier yielded full sensitivity and specificity in detecting subjects with Rahman syndrome. Applying this model to a cohort of undiagnosed probands allowed us to reach diagnosis in one subject. We demonstrate an epigenetic signature in subjects with Rahman syndrome that can be used to reach molecular diagnosis.

Sections du résumé

BACKGROUND
We previously associated HIST1H1E mutations causing Rahman syndrome with a specific genome-wide methylation pattern.
RESULTS
Methylome analysis from peripheral blood samples of six affected subjects led us to identify a specific hypomethylated profile. This "episignature" was enriched for genes involved in neuronal system development and function. A computational classifier yielded full sensitivity and specificity in detecting subjects with Rahman syndrome. Applying this model to a cohort of undiagnosed probands allowed us to reach diagnosis in one subject.
CONCLUSIONS
We demonstrate an epigenetic signature in subjects with Rahman syndrome that can be used to reach molecular diagnosis.

Identifiants

pubmed: 31910894
doi: 10.1186/s13148-019-0804-0
pii: 10.1186/s13148-019-0804-0
pmc: PMC6947958
doi:

Substances chimiques

H1-4 protein, human 0
Histones 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

7

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Auteurs

Andrea Ciolfi (A)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, IRCCS, 00146, Rome, Italy.

Erfan Aref-Eshghi (E)

Department of Pathology and Laboratory Medicine, Western University, London, N6A 5C1, Canada.
Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, N6A 5W9, Canada.

Simone Pizzi (S)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, IRCCS, 00146, Rome, Italy.

Lucia Pedace (L)

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

Evelina Miele (E)

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

Jennifer Kerkhof (J)

Department of Pathology and Laboratory Medicine, Western University, London, N6A 5C1, Canada.
Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, N6A 5W9, Canada.

Elisabetta Flex (E)

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

Simone Martinelli (S)

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

Francesca Clementina Radio (FC)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, IRCCS, 00146, Rome, Italy.

Claudia A L Ruivenkamp (CAL)

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

Gijs W E Santen (GWE)

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

Emilia Bijlsma (E)

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

Daniela Barge-Schaapveld (D)

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

Katrin Ounap (K)

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

Victoria Mok Siu (VM)

Medical Genetics Program of Southwestern Ontario, London Health Sciences Centre, London, ON, N6A 5W9, Canada.

R Frank Kooy (RF)

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

Bruno Dallapiccola (B)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, IRCCS, 00146, Rome, Italy.

Bekim Sadikovic (B)

Department of Pathology and Laboratory Medicine, Western University, London, N6A 5C1, Canada. bekim.sadikovic@lhsc.on.ca.
Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, N6A 5W9, Canada. bekim.sadikovic@lhsc.on.ca.

Marco Tartaglia (M)

Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, IRCCS, 00146, Rome, Italy. marco.tartaglia@opbg.net.

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