Literature DB >> 16450350

Molecular characterisation of a prenatally diagnosed 5q15q21.3 deletion and review of the literature.

Valérie Malan1, Jelena Martinovic, Damien Sanlaville, Stéphanie Caillat, Marie-Christine Perrier Waill, Marie-Laure Maurin Ganne, Julia Tantau, Tania Attie-Bitach, Michel Vekemans, Nicole Morichon-Delvallez.   

Abstract

BACKGROUND: Ultrasound examination performed on a 32-year old woman at 30 weeks' gestation showed the presence of fetal malformations. Amniocentesis was performed. METHODS AND
RESULTS: Cytogenetic analysis of cultured amniocytes revealed an interstitial deletion of the long arm of chromosome 5. Molecular studies confirmed that the deletion included the 5q15-21.3 region and was 14 Mb in size. Therefore, the karyotype was: 46,XY,del(5)(q15q21.3). In addition, analysis of polymorphic DNA markers showed that the deletion was of paternal origin.
CONCLUSIONS: The pregnancy was terminated at 34 weeks' gestation. At autopsy, the fetus displayed dysmorphic features, thin limbs and renal abnormalities. The clinical findings observed in the fetus as well as in 20 cases reported previously allowed us to further delineate the phenotype of such interstitial 5q15q21.3 deletions. 2006 John Wiley & Sons, Ltd.

Entities:  

Mesh:

Year:  2006        PMID: 16450350     DOI: 10.1002/pd.1386

Source DB:  PubMed          Journal:  Prenat Diagn        ISSN: 0197-3851            Impact factor:   3.050


  3 in total

1.  MEF2C haploinsufficiency caused by either microdeletion of the 5q14.3 region or mutation is responsible for severe mental retardation with stereotypic movements, epilepsy and/or cerebral malformations.

Authors:  N Le Meur; M Holder-Espinasse; S Jaillard; A Goldenberg; S Joriot; P Amati-Bonneau; A Guichet; M Barth; A Charollais; H Journel; S Auvin; C Boucher; J-P Kerckaert; V David; S Manouvrier-Hanu; P Saugier-Veber; T Frébourg; C Dubourg; J Andrieux; D Bonneau
Journal:  J Med Genet       Date:  2009-07-09       Impact factor: 6.318

2.  Interstitial deletion 5q14.3-q21 associated with iris coloboma, hearing loss, dental anomaly, moderate intellectual disability, and attention deficit and hyperactivity disorder.

Authors:  Nara Sobreira; Michael F Walsh; Denise Batista; Tao Wang
Journal:  Am J Med Genet A       Date:  2009-11       Impact factor: 2.802

3.  The novel lncRNA lnc-NR2F1 is pro-neurogenic and mutated in human neurodevelopmental disorders.

Authors:  Cheen Euong Ang; Qing Ma; Orly L Wapinski; Anand Srivastava; Marius Wernig; Howard Y Chang; ShengHua Fan; Ryan A Flynn; Qian Yi Lee; Bradley Coe; Masahiro Onoguchi; Victor Hipolito Olmos; Brian T Do; Lynn Dukes-Rimsky; Jin Xu; Koji Tanabe; LiangJiang Wang; Ulrich Elling; Josef M Penninger; Yang Zhao; Kun Qu; Evan E Eichler
Journal:  Elife       Date:  2019-01-10       Impact factor: 8.140

  3 in total

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