CMMB 403 Lecture Notes - Lecture 6: Rett Syndrome, Nucleosome, Growth Factor

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Mecp2 is a methylated cytosine binding protein. Its purpose is to recruit proteins for silenced genes, and to recruit histone deacetylases (hdac)1 so that the chromatin becomes compact again and the gene becomes silenced. Can also recruit histone methyltransferases (hmt) which add methyl groups to the histones. Both modifications (addition of hdac or hmt) promote the stability of the nucleosome and the tight packing of dna, thereby repressing gene expression in these regions of dna methylation. Mutations of mecp2 cause rett syndrome, which is a severe debilitating neurological disorder affecting girls. It involves methylation on the x chromosome, which females have 2 copies of and males only 1. The gene is found on the x chromosome. Males are affected worse if they don"t have the gene, or it is activated, the embryo dies. Nervous system and brain gets really small. The (cid:1688)de novo(cid:1689) methyltransferase dnmt3 can place a methyl group on unmethylated cytosines.

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