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A t(7;12) balanced translocation with breakpoints overlapping those of the Williams-Beuren and 12q14 microdeletion syndromes.
Gimelli, Stefania; Chrast, Jacqueline; Baban, Anwar; Henrichsen, Charlotte N; Lerone, Margherita; Zuffardi, Orsetta; Gimelli, Giorgio; Reymond, Alexandre.
Afiliación
  • Gimelli S; Biologia Generale e Genetica Medica, Universita' di Pavia and IRCCS C. Mondino, Pavia, Italy. stefania.gimelli@gmail.com
Am J Med Genet A ; 152A(5): 1285-94, 2010 May.
Article en En | MEDLINE | ID: mdl-20425838
The molecular characterization of balanced chromosomal rearrangements have always been of advantage in identifying disease-causing genes. Here, we describe the breakpoint mapping of a de novo balanced translocation t(7;12)(q11.22;q14.2) in a patient presenting with a failure to thrive associated with moderate mental retardation, facial anomalies, and chronic constipation. The localization of the breakpoints and the co-occurrence of Williams-Beuren syndrome and 12q14 microdeletion syndrome phenotypes suggested that the expression of some of the dosage-sensitive genes of these two segmental aneuploidies were modified in cells of the proposita. However, we were unable to identify chromosomes 7 and/or 12-mapping genes that showed disturbed expression in the lymphoblastoids of the proposita. This case showed that position-effect might operate in some tissues, but not in others. It also illustrates the overlap of phenotypes presented by patients with the recently described 12q14 structural rearrangements.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Translocación Genética / Cromosomas Humanos Par 7 / Cromosomas Humanos Par 12 / Deleción Cromosómica / Síndrome de Williams / Rotura Cromosómica Tipo de estudio: Prognostic_studies Límite: Female / Humans / Infant / Newborn / Pregnancy Idioma: En Revista: Am J Med Genet A Asunto de la revista: GENETICA MEDICA Año: 2010 Tipo del documento: Article País de afiliación: Italia

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Translocación Genética / Cromosomas Humanos Par 7 / Cromosomas Humanos Par 12 / Deleción Cromosómica / Síndrome de Williams / Rotura Cromosómica Tipo de estudio: Prognostic_studies Límite: Female / Humans / Infant / Newborn / Pregnancy Idioma: En Revista: Am J Med Genet A Asunto de la revista: GENETICA MEDICA Año: 2010 Tipo del documento: Article País de afiliación: Italia