Literature DB >> 25701697

Warsaw Breakage Syndrome--A further report, emphasising cutaneous findings.

Claire Bailey1, Alan E Fryer2, Mark Greenslade3.   

Abstract

We report a new case of Warsaw Breakage syndrome (WABS) with 2 confirmed mutations in DDX11. Like the previous reported cases [Capo-Chichi et al., 2012; Van der Lelij et al., 2010], there was evidence of pre- and postnatal growth retardation, severe microcephaly, intellectual disability and facial dysmorphism. The patient had sensorineural hearing loss with evidence of bilateral hypoplastic cochleas on imaging, another feature which has been reported in the previous cases of WABS. In our case the patient exhibited a chronic rash of livedo reticularis with telangiectasia on her legs. Abnormally pigmented lesions and cutis mamorata were reported in the original WABS case.
Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Entities:  

Keywords:  Cohesinopathy; Cutaneous; DDX11; Warsaw Breakage Syndrome (WABS)

Mesh:

Substances:

Year:  2015        PMID: 25701697     DOI: 10.1016/j.ejmg.2015.02.001

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


  8 in total

1.  Warsaw breakage syndrome: Further clinical and genetic delineation.

Authors:  Ebba Alkhunaizi; Ranad Shaheen; Sanjay Kumar Bharti; Ann M Joseph-George; Karen Chong; Ghada M H Abdel-Salam; Mohammed Alowain; Susan I Blaser; Blake C Papsin; Mohammed Butt; Mais Hashem; Nicole Martin; Ruth Godoy; Robert M Brosh; Fowzan S Alkuraya; David Chitayat
Journal:  Am J Med Genet A       Date:  2018-09-14       Impact factor: 2.802

2.  Warsaw Breakage Syndrome associated DDX11 helicase resolves G-quadruplex structures to support sister chromatid cohesion.

Authors:  Janne J M van Schie; Atiq Faramarz; Jesper A Balk; Grant S Stewart; Erika Cantelli; Anneke B Oostra; Martin A Rooimans; Joanna L Parish; Cynthia de Almeida Estéves; Katja Dumic; Ingeborg Barisic; Karin E M Diderich; Marjon A van Slegtenhorst; Mohammad Mahtab; Francesca M Pisani; Hein Te Riele; Najim Ameziane; Rob M F Wolthuis; Job de Lange
Journal:  Nat Commun       Date:  2020-08-27       Impact factor: 14.919

3.  The Q Motif Is Involved in DNA Binding but Not ATP Binding in ChlR1 Helicase.

Authors:  Hao Ding; Manhong Guo; Venkatasubramanian Vidhyasagar; Tanu Talwar; Yuliang Wu
Journal:  PLoS One       Date:  2015-10-16       Impact factor: 3.240

4.  Warsaw breakage syndrome DDX11 helicase acts jointly with RAD17 in the repair of bulky lesions and replication through abasic sites.

Authors:  Takuya Abe; Masato Ooka; Ryotaro Kawasumi; Keiji Miyata; Minoru Takata; Kouji Hirota; Dana Branzei
Journal:  Proc Natl Acad Sci U S A       Date:  2018-07-30       Impact factor: 11.205

Review 5.  Molecular and Cellular Functions of the Warsaw Breakage Syndrome DNA Helicase DDX11.

Authors:  Francesca M Pisani; Ettore Napolitano; Luisa M R Napolitano; Silvia Onesti
Journal:  Genes (Basel)       Date:  2018-11-21       Impact factor: 4.096

6.  Heterogeneous clinical phenotypes and cerebral malformations reflected by rotatin cellular dynamics.

Authors:  Laura V Vandervore; Rachel Schot; Esmee Kasteleijn; Renske Oegema; Katrien Stouffs; Alexander Gheldof; Martyna M Grochowska; Marianne L T van der Sterre; Leontine M A van Unen; Martina Wilke; Peter Elfferich; Peter J van der Spek; Daphne Heijsman; Anna Grandone; Jeroen A A Demmers; Dick H W Dekkers; Johan A Slotman; Gert-Jan Kremers; Gerben J Schaaf; Roy G Masius; Anton J van Essen; Patrick Rump; Arie van Haeringen; Els Peeters; Umut Altunoglu; Tugba Kalayci; Raymond A Poot; William B Dobyns; Nadia Bahi-Buisson; Frans W Verheijen; Anna C Jansen; Grazia M S Mancini
Journal:  Brain       Date:  2019-04-01       Impact factor: 13.501

7.  Two further patients with Warsaw breakage syndrome. Is a mild phenotype possible?

Authors:  Roberta Bottega; Luisa M R Napolitano; Anna Carbone; Enrico Cappelli; Fabio Corsolini; Silvia Onesti; Anna Savoia; Paolo Gasparini; Flavio Faletra
Journal:  Mol Genet Genomic Med       Date:  2019-03-28       Impact factor: 2.183

8.  The iron-sulfur helicase DDX11 promotes the generation of single-stranded DNA for CHK1 activation.

Authors:  Anna K Simon; Sandra Kummer; Sebastian Wild; Aleksandra Lezaja; Federico Teloni; Stanislaw K Jozwiakowski; Matthias Altmeyer; Kerstin Gari
Journal:  Life Sci Alliance       Date:  2020-02-18
  8 in total

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