Literature DB >> 16451141

Adams-Oliver syndrome: clinical description of a four-generation family and exclusion of five candidate genes.

P Verdyck1, B Blaumeiser, M Holder-Espinasse, W Van Hul, W Wuyts.   

Abstract

We present a Belgian Adams-Oliver syndrome (AOS) family with 10 affected individuals over four generations, of which six were available for this study. Clinical symptoms observed in these patients were very variable as previously reported in other families and included large areas of alopecia on the vertex of the skull and serious limb reduction defects with agenesis of all toes of one foot. To identify the disease-causing gene, we sequenced the MSX1, CART1, P63 (P73L), RUNX2, and HOXD13 genes in this family and nine previously reported families, but no disease-causing mutations were found. Further investigation is ongoing in these families in order to identify the genetic cause of AOS.

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Year:  2006        PMID: 16451141     DOI: 10.1111/j.1399-0004.2006.00552.x

Source DB:  PubMed          Journal:  Clin Genet        ISSN: 0009-9163            Impact factor:   4.438


  4 in total

1.  Gain-of-function mutations of ARHGAP31, a Cdc42/Rac1 GTPase regulator, cause syndromic cutis aplasia and limb anomalies.

Authors:  Laura Southgate; Rajiv D Machado; Katie M Snape; Martin Primeau; Dimitra Dafou; Deborah M Ruddy; Peter A Branney; Malcolm Fisher; Grace J Lee; Michael A Simpson; Yi He; Teisha Y Bradshaw; Bettina Blaumeiser; William S Winship; Willie Reardon; Eamonn R Maher; David R FitzPatrick; Wim Wuyts; Martin Zenker; Nathalie Lamarche-Vane; Richard C Trembath
Journal:  Am J Hum Genet       Date:  2011-05-13       Impact factor: 11.025

2.  A case of adams-oliver syndrome.

Authors:  Jong-Keun Seo; Ju-Hyun Kang; Hyun-Jae Lee; Deborah Lee; Ho-Suk Sung; Seon-Wook Hwang
Journal:  Ann Dermatol       Date:  2010-02-28       Impact factor: 1.444

3.  Dilemmas and challenges in the management of a neonate with Adams-Oliver syndrome with infected giant aplasia cutis lesion and exsanguination: a case-based update.

Authors:  Suhas Udayakumaran; Jimmy Mathew; Dilip Panikar
Journal:  Childs Nerv Syst       Date:  2012-12-29       Impact factor: 1.475

4.  Elucidating the genetic architecture of Adams-Oliver syndrome in a large European cohort.

Authors:  Josephina A N Meester; Maja Sukalo; Kim C Schröder; Denny Schanze; Gareth Baynam; Guntram Borck; Nuria C Bramswig; Duygu Duman; Brigitte Gilbert-Dussardier; Muriel Holder-Espinasse; Peter Itin; Diana S Johnson; Shelagh Joss; Hannele Koillinen; Fiona McKenzie; Jenny Morton; Heike Nelle; Willie Reardon; Claudia Roll; Mustafa A Salih; Ravi Savarirayan; Ingrid Scurr; Miranda Splitt; Elizabeth Thompson; Hannah Titheradge; Colm P Travers; Lionel Van Maldergem; Margo Whiteford; Dagmar Wieczorek; Geert Vandeweyer; Richard Trembath; Lut Van Laer; Bart L Loeys; Martin Zenker; Laura Southgate; Wim Wuyts
Journal:  Hum Mutat       Date:  2018-07-04       Impact factor: 4.878

  4 in total

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