Literature DB >> 7717414

Deletion of chromosome 2q24-q31 causes characteristic digital anomalies: case report and review.

R G Boles1, B R Pober, L H Gibson, C R Willis, J McGrath, D J Roberts, T L Yang-Feng.   

Abstract

We describe a newborn boy with multiple anomalies, including bilateral split foot and an interstitial deletion of chromosome 2 (q24.2-q31.1). Four additional cases in 2 families involving similar deletions have been reported. Bilateral digital anomalies of hands and feet were seen in all 5 cases, including a wide cleft between the first and second toes, wide halluces, brachysyndactyly of the toes, and camptodactyly of the fingers. Other common manifestations have included postnatal growth and mental retardation, microcephaly, down-slanting palpebral fissures, micrognathia, and apparently low-set ears. Bilateral digital anomalies were reported in 22 of 24 cases with deletions including at least part of region 2q24-q31. Digital anomalies were not prevalent in 18 patients with deletions of chromosome 2q not overlapping 2q24-q31. 2q31.1 appears to be the common deleted segment in all cases with significant digital anomalies, which implies the existence of one or more genes involved in distal limb morphogenesis in this region. HOXD13 and EVX2, located in the proximity of 2q31, were not deleted in our patient by Southern analysis. Bilateral digital malformations of the hands and feet associated with other anomalies should be evaluated by chromosome analysis focused at the 2q24-q31 region.

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Year:  1995        PMID: 7717414     DOI: 10.1002/ajmg.1320550204

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  17 in total

1.  Split-hand/split-foot malformation is caused by mutations in the p63 gene on 3q27.

Authors:  P Ianakiev; M W Kilpatrick; I Toudjarska; D Basel; P Beighton; P Tsipouras
Journal:  Am J Hum Genet       Date:  2000-06-05       Impact factor: 11.025

2.  Refinement of the Region for Split Hand/Foot Malformation 5 on 2q31.1.

Authors:  A Theisen; J A Rosenfeld; K Shane; K L McBride; J F Atkin; C Gaba; J Hoo; T W Kurczynski; R E Schnur; L B Coffey; E H Zackai; L Schimmenti; N Friedman; M Zabukovec; S Ball; R Pagon; A Lucas; C K Brasington; J E Spence; S Sparks; V Banks; W Smith; T Friedberg; P R Wyatt; M Aust; R Tervo; A Crowley; D Skidmore; A N Lamb; B Ravnan; T Sahoo; R Schultz; B S Torchia; M Sgro; D Chitayat; L G Shaffer
Journal:  Mol Syndromol       Date:  2011-05-18

3.  Sorting nexin 3 (SNX3) is disrupted in a patient with a translocation t(6;13)(q21;q12) and microcephaly, microphthalmia, ectrodactyly, prognathism (MMEP) phenotype.

Authors:  V S Vervoort; D Viljoen; R Smart; G Suthers; B R DuPont; A Abbott; C E Schwartz
Journal:  J Med Genet       Date:  2002-12       Impact factor: 6.318

4.  Nonsyndromic Split-Hand/Foot Malformation: Recent Classification.

Authors:  Muhammad Umair; Amir Hayat
Journal:  Mol Syndromol       Date:  2019-09-18

5.  Monodactylous limbs and abnormal genitalia are associated with hemizygosity for the human 2q31 region that includes the HOXD cluster.

Authors:  M Del Campo; M C Jones; A N Veraksa; C J Curry; K L Jones; J T Mascarello; Z Ali-Kahn-Catts; T Drumheller; W McGinnis
Journal:  Am J Hum Genet       Date:  1999-07       Impact factor: 11.025

6.  Familial syndromic esophageal atresia maps to 2p23-p24.

Authors:  J Celli; E van Beusekom; R C Hennekam; M E Gallardo; D F Smeets; S R de Córdoba; J W Innis; M Frydman; R König; H Kingston; J Tolmie; L C Govaerts; H van Bokhoven; H G Brunner
Journal:  Am J Hum Genet       Date:  2000-02       Impact factor: 11.025

7.  A 117-kb microdeletion removing HOXD9-HOXD13 and EVX2 causes synpolydactyly.

Authors:  Frances R Goodman; Frank Majewski; Amanda L Collins; Peter J Scambler
Journal:  Am J Hum Genet       Date:  2002-01-03       Impact factor: 11.025

8.  Breakpoints around the HOXD cluster result in various limb malformations.

Authors:  B Dlugaszewska; A Silahtaroglu; C Menzel; S Kübart; M Cohen; S Mundlos; Z Tümer; K Kjaer; U Friedrich; H-H Ropers; N Tommerup; H Neitzel; V M Kalscheuer
Journal:  J Med Genet       Date:  2005-06-24       Impact factor: 6.318

9.  Genomic rearrangement at 10q24 in non-syndromic split-hand/split-foot malformation.

Authors:  Hiroki Kano; Kenji Kurosawa; Emiko Horii; Shiro Ikegawa; Hideki Yoshikawa; Hiroki Kurahashi; Tatsushi Toda
Journal:  Hum Genet       Date:  2005-10-19       Impact factor: 4.132

10.  Dlx5 Homeodomain:DNA Complex: Structure, Binding and Effect of Mutations Related to Split Hand and Foot Malformation Syndrome.

Authors:  Andrew Proudfoot; Herbert L Axelrod; Michael Geralt; Robert J Fletterick; Fumiaki Yumoto; Ashley M Deacon; Marc-André Elsliger; Ian A Wilson; Kurt Wüthrich; Pedro Serrano
Journal:  J Mol Biol       Date:  2016-01-29       Impact factor: 5.469

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