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Example sentences for: rett
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Previously reported microarray studies of female Rett and unrelated control brain samples revealed expression changes that were interpreted as secondary to the disease process, such as increased expression of glial-specific genes and decreased expression of neuronal genes [ 25 ] . Therefore, when searching for the primary targets of MeCP2 modulation, it might be advantageous to study a tissue that is less obviously involved in the pathologic processes of the disease.
The use of clonally derived fibroblasts eliminated the mosaic X inactivation seen in tissues from most females with Rett syndrome.
To identify potential downstream targets of MeCP2 modulation, we have compared global gene expression patterns in four matched pairs of clonally derived mutant or wild-type MECP2-expressing fibroblast cell strains from girls with Rett syndrome, and in five wild-type and four mutant MeCP2-expressing lymphoblastoid cell lines, by hybridizing Affymetrix U95 (A-E) oligonucleotide microarrays.
Clonal cell lines with the mutant MECP2 allele on the active X chromosome were derived from lymphoblastoid cell lines (LCLs) from four females with clinically diagnosed Rett syndrome and known mutations (T158M, 803delG, R168X) [ 10 ] and 1159del28 (unpublished) using the technique described above.
Our study design was intended to take advantage of the mosaic X chromosome inactivation in cell strains of Rett syndrome females.
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