Literature DB >> 2786338

Incidence of 15q deletions in the Angelman syndrome: a survey of twelve affected persons.

C A Williams1, B A Gray, J E Hendrickson, J W Stone, E S Cantú.   

Abstract

Prometaphase chromosome study of 12 persons with an established diagnosis of the Angelman syndrome demonstrated that 5 had a 15q12 deletion appearing similar to that commonly observed in the Prader-Willi syndrome. Phenotype-karyotype correlation did not show any obvious clinical differences between those with and those without the deletion and no clinical overlap between Angelman and Prader-Willi syndrome was apparent. Our survey suggests that 15q12 deletions are frequent in Angelman syndrome but presence of the deletion does not appear to distinguish different clinical phenotypes. Experience with the cytogenetic study of Prader-Willi syndrome predicts that considerable complexity will emerge between the presence of 15 chromosome abnormalities and clinical expression of Angelman syndrome.

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Year:  1989        PMID: 2786338     DOI: 10.1002/ajmg.1320320313

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


  21 in total

Review 1.  Chromosome imbalance, normal phenotype, and imprinting.

Authors:  L Bortotto; E Piovan; R Furlan; H Rivera; O Zuffardi
Journal:  J Med Genet       Date:  1990-09       Impact factor: 6.318

2.  Decrease in benzodiazepine receptor binding in a patient with Angelman syndrome detected by iodine-123 iomazenil and single-photon emission tomography.

Authors:  I Odano; T Anezaki; M Ohkubo; Y Yonekura; Y Onishi; T Inuzuka; M Takahashi; S Tsuji
Journal:  Eur J Nucl Med       Date:  1996-05

3.  Angelman's syndrome: a neuropathological study.

Authors:  T Kyriakides; L A Hallam; A Hockey; P Silberstein; B A Kakulas
Journal:  Acta Neuropathol       Date:  1992       Impact factor: 17.088

4.  Angelman syndrome.

Authors:  J Clayton-Smith; M E Pembrey
Journal:  J Med Genet       Date:  1992-06       Impact factor: 6.318

5.  Angelman syndrome in an inbred family.

Authors:  J Beuten; R C Hennekam; B Van Roy; K Mangelschots; J S Sutcliffe; D J Halley; F A Hennekam; A L Beaudet; P J Willems
Journal:  Hum Genet       Date:  1996-03       Impact factor: 4.132

6.  Elevated plasma gamma-aminobutyric acid (GABA) levels in individuals with either Prader-Willi syndrome or Angelman syndrome.

Authors:  M H Ebert; D E Schmidt; T Thompson; M G Butler
Journal:  J Neuropsychiatry Clin Neurosci       Date:  1997       Impact factor: 2.198

Review 7.  Clinical features in 27 patients with Angelman syndrome resulting from DNA deletion.

Authors:  A Smith; C Wiles; E Haan; J McGill; G Wallace; J Dixon; R Selby; A Colley; R Marks; R J Trent
Journal:  J Med Genet       Date:  1996-02       Impact factor: 6.318

8.  The frequency of uniparental disomy in Prader-Willi syndrome. Implications for molecular diagnosis.

Authors:  M J Mascari; W Gottlieb; P K Rogan; M G Butler; D A Waller; J A Armour; A J Jeffreys; R L Ladda; R D Nicholls
Journal:  N Engl J Med       Date:  1992-06-11       Impact factor: 91.245

9.  Molecular, cytogenetic, and clinical investigations of Prader-Willi syndrome patients.

Authors:  W P Robinson; A Bottani; Y G Xie; J Balakrishman; F Binkert; M Mächler; A Prader; A Schinzel
Journal:  Am J Hum Genet       Date:  1991-12       Impact factor: 11.025

10.  Multiplex PCR of three dinucleotide repeats in the Prader-Willi/Angelman critical region (15q11-q13): molecular diagnosis and mechanism of uniparental disomy.

Authors:  A Mutirangura; F Greenberg; M G Butler; S Malcolm; R D Nicholls; A Chakravarti; D H Ledbetter
Journal:  Hum Mol Genet       Date:  1993-02       Impact factor: 6.150

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