A t(7;12) balanced translocation with breakpoints overlapping those of the Williams-Beuren and 12q14 microdeletion syndromes.
Data(s) |
2010
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Resumo |
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. |
Identificador |
https://serval.unil.ch/?id=serval:BIB_078F653B401C isbn:1552-4833[electronic], 1552-4825[linking] pmid:20425838 doi:10.1002/ajmg.a.33365 isiid:000277739800028 |
Idioma(s) |
en |
Fonte |
American Journal of Medical Genetics. Part A, vol. 152A, no. 5, pp. 1285-1294 |
Palavras-Chave | #Cell Line; Chromosome Banding; Chromosome Breakage; Chromosome Deletion; Chromosome Mapping; Chromosomes, Human, Pair 12/genetics; Chromosomes, Human, Pair 7/genetics; Comparative Genomic Hybridization; Female; Humans; Infant; Infant, Newborn; Karyotyping; Pregnancy; Translocation, Genetic; Williams Syndrome/genetics |
Tipo |
info:eu-repo/semantics/article article |