A t(7;12) balanced translocation with breakpoints overlapping those of the Williams-Beuren and 12q14 microdeletion syndromes.


Autoria(s): Gimelli S.; Chrast J.; Baban A.; Henrichsen C.N.; Lerone M.; Zuffardi O.; Gimelli G.; Reymond A.
Data(s)

2010

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