Literature DB >> 30986546

Further delineation of CDC45-related Meier-Gorlin syndrome with craniosynostosis and review of literature.

Chun Yi Ting1, Neha Singh Bhatia2, Jiin Ying Lim3, Chew-Yin Jasmine Goh4, Rashida Farhad Vasanwala5, Caroline Choo-Phaik Ong6, Wan Tew Seow7, Vincent Kok-Leng Yeow8, Teck Wah Ting9, Ivy Swee-Lian Ng10, Saumya Shekhar Jamuar11.   

Abstract

Meier-Gorlin syndrome (MGS) is a rare autosomal recessive disorder characterized by the triad of short stature, microtia and absent or small patellae. We report on a patient with MGS secondary to biallelic mutations in CDC45 detected on whole exome sequencing (WES). Patients with MGS caused by mutations in CDC45 display a distinct phenotype characterized by craniosynostosis and anorectal malformation. Our patient had craniosynostosis, anorectal malformation and short stature, but did not have the microtia or patella hypoplasia. Our report also highlights the value of WES in aiding diagnosis of patients with rare genetic diseases. In conclusion, our case report and review of the literature illustrates the unique features of CDC45-related MGS as well as the benefits of WES in reducing the diagnostic odyssey for patients with rare genetic disorders.
Copyright © 2019 Elsevier Masson SAS. All rights reserved.

Entities:  

Keywords:  Craniosynostosis; Diagnostic odyssey; Meier-Gorlin syndrome; anorectal malformations; whole exome sequencing

Year:  2019        PMID: 30986546     DOI: 10.1016/j.ejmg.2019.04.009

Source DB:  PubMed          Journal:  Eur J Med Genet        ISSN: 1769-7212            Impact factor:   2.708


  7 in total

1.  Hypomorphic GINS3 variants alter DNA replication and cause Meier-Gorlin syndrome.

Authors:  Mary E McQuaid; Kashif Ahmed; Stephanie Tran; Justine Rousseau; Ranad Shaheen; Kristin D Kernohan; Kyoko E Yuki; Prerna Grover; Ema S Dreseris; Sameen Ahmed; Lucie Dupuis; Jennifer Stimec; Mary Shago; Zuhair N Al-Hassnan; Roch Tremblay; Philipp G Maass; Michael D Wilson; Eyal Grunebaum; Kym M Boycott; François-Michel Boisvert; Sateesh Maddirevula; Eissa A Faqeih; Fahad Almanjomi; Zaheer Ullah Khan; Fowzan S Alkuraya; Philippe M Campeau; Peter Kannu; Eric I Campos; Hugo Wurtele
Journal:  JCI Insight       Date:  2022-05-23

2.  High abundance of CDC45 inhibits cell proliferation through elevation of HSPA6.

Authors:  Yuanyuan Fu; Zhiyi Lv; Deqing Kong; Yuping Fan; Bo Dong
Journal:  Cell Prolif       Date:  2022-06-01       Impact factor: 8.755

Review 3.  Congenital Diseases of DNA Replication: Clinical Phenotypes and Molecular Mechanisms.

Authors:  Megan Schmit; Anja-Katrin Bielinsky
Journal:  Int J Mol Sci       Date:  2021-01-18       Impact factor: 5.923

4.  Novel Compound Heterozygous Variants in the CDC6 Gene in a Russian Patient with Meier-Gorlin Syndrome.

Authors:  Viktoriia Zabnenkova; Olga Shchagina; Olga Makienko; Galina Matyushchenko; Oxana Ryzhkova
Journal:  Appl Clin Genet       Date:  2022-01-06

5.  Meier-Gorlin syndrome with prenatal ultrasound findings and successful growth hormone therapy: Six years follow-up of a rare case.

Authors:  Rahim Vakili; Moein Mobini; Farbod Hatami; Saba Vakili; Niloufar Valizadeh
Journal:  Radiol Case Rep       Date:  2022-03-04

6.  De novo 22q11.2 deletions and auricular findings in two Chinese patients with microtia.

Authors:  Nuo Si; Zeya Zhang; Xin Huang; Chanchen Wang; Peipei Guo; Bo Pan; Haiyue Jiang
Journal:  Mol Genet Genomic Med       Date:  2021-12-31       Impact factor: 2.183

7.  An additional whole-exome sequencing study in 102 panel-undiagnosed patients: A retrospective study in a Chinese craniosynostosis cohort.

Authors:  Jieyi Chen; Ping Zhang; Meifang Peng; Bo Liu; Xiao Wang; Siyuan Du; Yao Lu; Xiongzheng Mu; Yulan Lu; Sijia Wang; Yingzhi Wu
Journal:  Front Genet       Date:  2022-09-02       Impact factor: 4.772

  7 in total

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