De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complications

Abstract Beckwith–Wiedemann spectrum (BWSp) is a genomic imprinting disorder characterized by a wide range of clinical features. Here we report an infant with BWSp and atypical features, for whom long-read sequencing confirmed a de novo CDKN1C variant that occurred on the maternally inherited allele...

Full description

Saved in:
Bibliographic Details
Main Authors: Yuri Moriura, Yosuke Nishio, Shintaro Ichimura, Haruka Noda, Yoshihiro Tanahashi, Hikaru Yamamoto, Yuka Nakazawa, Taichi Oso, Yoshiaki Sato, Toshiki Takenouchi, Shinji Saitoh, Yukako Muramatsu, Tomoo Ogi
Format: Article
Language:English
Published: Nature Publishing Group 2025-05-01
Series:Human Genome Variation
Online Access:https://doi.org/10.1038/s41439-025-00316-0
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849688368818946048
author Yuri Moriura
Yosuke Nishio
Shintaro Ichimura
Haruka Noda
Yoshihiro Tanahashi
Hikaru Yamamoto
Yuka Nakazawa
Taichi Oso
Yoshiaki Sato
Toshiki Takenouchi
Shinji Saitoh
Yukako Muramatsu
Tomoo Ogi
author_facet Yuri Moriura
Yosuke Nishio
Shintaro Ichimura
Haruka Noda
Yoshihiro Tanahashi
Hikaru Yamamoto
Yuka Nakazawa
Taichi Oso
Yoshiaki Sato
Toshiki Takenouchi
Shinji Saitoh
Yukako Muramatsu
Tomoo Ogi
author_sort Yuri Moriura
collection DOAJ
description Abstract Beckwith–Wiedemann spectrum (BWSp) is a genomic imprinting disorder characterized by a wide range of clinical features. Here we report an infant with BWSp and atypical features, for whom long-read sequencing confirmed a de novo CDKN1C variant that occurred on the maternally inherited allele and excluded other genetic etiologies. These findings not only expand the BWSp concept but also highlight the potential value of allelic origin analysis in cases with atypical presentations.
format Article
id doaj-art-98114c409ebf4d71b2f659f2a33e0d87
institution DOAJ
issn 2054-345X
language English
publishDate 2025-05-01
publisher Nature Publishing Group
record_format Article
series Human Genome Variation
spelling doaj-art-98114c409ebf4d71b2f659f2a33e0d872025-08-20T03:22:01ZengNature Publishing GroupHuman Genome Variation2054-345X2025-05-011211310.1038/s41439-025-00316-0De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complicationsYuri Moriura0Yosuke Nishio1Shintaro Ichimura2Haruka Noda3Yoshihiro Tanahashi4Hikaru Yamamoto5Yuka Nakazawa6Taichi Oso7Yoshiaki Sato8Toshiki Takenouchi9Shinji Saitoh10Yukako Muramatsu11Tomoo Ogi12Department of Pediatrics, Nagoya University Graduate School of MedicineDepartment of Pediatrics, Nagoya University Graduate School of MedicineDivision of Neonatology, Center for Maternal-Neonatal Care, Nagoya University HospitalDivision of Neonatology, Center for Maternal-Neonatal Care, Nagoya University HospitalDepartment of Pediatrics, TOYOTA Memorial HospitalDepartment of Pediatrics, TOYOTA Memorial HospitalDepartment of Genetics, Research Institute of Environmental Medicine, Nagoya UniversityDepartment of Genetics, Research Institute of Environmental Medicine, Nagoya UniversityDivision of Neonatology, Center for Maternal-Neonatal Care, Nagoya University HospitalDepartment of Pediatrics, Keio University School of MedicineDepartment of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical SciencesDepartment of Pediatrics, Nagoya University Graduate School of MedicineDepartment of Genetics, Research Institute of Environmental Medicine, Nagoya UniversityAbstract Beckwith–Wiedemann spectrum (BWSp) is a genomic imprinting disorder characterized by a wide range of clinical features. Here we report an infant with BWSp and atypical features, for whom long-read sequencing confirmed a de novo CDKN1C variant that occurred on the maternally inherited allele and excluded other genetic etiologies. These findings not only expand the BWSp concept but also highlight the potential value of allelic origin analysis in cases with atypical presentations.https://doi.org/10.1038/s41439-025-00316-0
spellingShingle Yuri Moriura
Yosuke Nishio
Shintaro Ichimura
Haruka Noda
Yoshihiro Tanahashi
Hikaru Yamamoto
Yuka Nakazawa
Taichi Oso
Yoshiaki Sato
Toshiki Takenouchi
Shinji Saitoh
Yukako Muramatsu
Tomoo Ogi
De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complications
Human Genome Variation
title De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complications
title_full De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complications
title_fullStr De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complications
title_full_unstemmed De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complications
title_short De novo CDKN1C variant in Beckwith–Wiedermann spectrum with atypical complications
title_sort de novo cdkn1c variant in beckwith wiedermann spectrum with atypical complications
url https://doi.org/10.1038/s41439-025-00316-0
work_keys_str_mv AT yurimoriura denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT yosukenishio denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT shintaroichimura denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT harukanoda denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT yoshihirotanahashi denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT hikaruyamamoto denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT yukanakazawa denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT taichioso denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT yoshiakisato denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT toshikitakenouchi denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT shinjisaitoh denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT yukakomuramatsu denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications
AT tomooogi denovocdkn1cvariantinbeckwithwiedermannspectrumwithatypicalcomplications