Homozygous mutation in


Journal

Journal of medical genetics
ISSN: 1468-6244
Titre abrégé: J Med Genet
Pays: England
ID NLM: 2985087R

Informations de publication

Date de publication:
05 2022
Historique:
received: 27 10 2020
revised: 15 02 2021
accepted: 02 03 2021
pubmed: 2 6 2021
medline: 27 4 2022
entrez: 1 6 2021
Statut: ppublish

Résumé

Minichromosomal maintenance (MCM) complex components 2, 4, 5 and 6 have been linked to human disease with phenotypes including microcephaly and intellectual disability. The MCM complex has DNA helicase activity and is thereby important for the initiation and elongation of the replication fork and highly expressed in proliferating neural stem cells. Whole-exome sequencing was applied to identify the genetic cause underlying the neurodevelopmental disease of the index family. The expression pattern of We reported that the homozygous missense variant c.793G>A/p.A265T (g.7:99695841C>T, NM_005916.4) in We report mutations of

Sections du résumé

BACKGROUND
Minichromosomal maintenance (MCM) complex components 2, 4, 5 and 6 have been linked to human disease with phenotypes including microcephaly and intellectual disability. The MCM complex has DNA helicase activity and is thereby important for the initiation and elongation of the replication fork and highly expressed in proliferating neural stem cells.
METHODS
Whole-exome sequencing was applied to identify the genetic cause underlying the neurodevelopmental disease of the index family. The expression pattern of
RESULTS
We reported that the homozygous missense variant c.793G>A/p.A265T (g.7:99695841C>T, NM_005916.4) in
CONCLUSION
We report mutations of

Identifiants

pubmed: 34059554
pii: jmedgenet-2020-107518
doi: 10.1136/jmedgenet-2020-107518
pmc: PMC9046757
doi:

Substances chimiques

MCM7 protein, human EC 3.6.4.12
Minichromosome Maintenance Complex Component 7 EC 3.6.4.12

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Pagination

453-461

Informations de copyright

© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

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Auteurs

Ethiraj Ravindran (E)

Institute of Cell Biology and Neurobiology, Charité Universitätsmedizin Berlin, Berlin, Germany.
Department of Pediatric Neurology, Charité Universitätsmedizin Berlin, Berlin, Germany.
Center for Chronically Sick Children (Sozialpädiatrisches Zentrum, SPZ), Charité - Universitätsmedizin Berlin, Berlin, Germany.

Cynthia Gutierrez de Velazco (C)

Institute of Cell Biology and Neurobiology, Charité Universitätsmedizin Berlin, Berlin, Germany.
Department of Pediatric Neurology, Charité Universitätsmedizin Berlin, Berlin, Germany.
Center for Chronically Sick Children (Sozialpädiatrisches Zentrum, SPZ), Charité - Universitätsmedizin Berlin, Berlin, Germany.

Ali Ghazanfar (A)

Department of Biotechnology, University of Azad Jammu and Kashmir, Muzaffarabad, Pakistan.

Nadine Kraemer (N)

Institute of Cell Biology and Neurobiology, Charité Universitätsmedizin Berlin, Berlin, Germany.
Department of Pediatric Neurology, Charité Universitätsmedizin Berlin, Berlin, Germany.
Center for Chronically Sick Children (Sozialpädiatrisches Zentrum, SPZ), Charité - Universitätsmedizin Berlin, Berlin, Germany.

Sami Zaqout (S)

Department of Basic Medical Sciences, College of Medicine, QU Health, Qatar University, Doha, Qatar.
Biomedical and Pharmaceutical Research Unit, QU Health, Qatar University, Doha, Qatar.

Abdul Waheed (A)

Department of Biotechnology, University of Azad Jammu and Kashmir, Muzaffarabad, Pakistan.

Mohsan Hanif (M)

Department of Biotechnology, University of Azad Jammu and Kashmir, Muzaffarabad, Pakistan.

Sadia Mughal (S)

Department of Biotechnology, University of Azad Jammu and Kashmir, Muzaffarabad, Pakistan.

Alessandro Prigione (A)

University Children's Hospital, Department of General Pediatrics, Heinrich-Heine-Universitat Dusseldorf, Düsseldorf, Germany.

Na Li (N)

Laboratory of Medical Systems Biology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.

Xiang Fang (X)

Laboratory of Medical Systems Biology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.

Hao Hu (H)

Laboratory of Medical Systems Biology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
Guangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
School of Medicine, South China University of Technology, Guangzhou, China.

Angela M Kaindl (AM)

Institute of Cell Biology and Neurobiology, Charité Universitätsmedizin Berlin, Berlin, Germany angela.kaindl@charite.de.
Department of Pediatric Neurology, Charité Universitätsmedizin Berlin, Berlin, Germany.
Center for Chronically Sick Children (Sozialpädiatrisches Zentrum, SPZ), Charité - Universitätsmedizin Berlin, Berlin, Germany.

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