Second human pseudoautosomal region: biology, genes and implications for the pathogenesis of Rett syndrome

Reference:
E.0869

Abstract:
Our interest is to clarify the biology of the second human pseudoautosomal region, located at the end of long arm of the X chromosome and its involvment in genetic pathologies. Genes located in this region show different transcriptional behaviour, escaping or not the X inactivation. Such effects could be likely caused by epigenetic factors and/or position effect variegation. The XqPAR may be thus a model region for studying these mechanisms in aetiology of genetic diseases. XQPAR genes seem also to be involved in the Rett syndrome, a neurological disorder recently linked to the distal Xq28 region. Our working hypothesis also follows a genetic model which explains the female selectivity of this disease, and directly involves genes we identified in the XqPAR.

PROJECT DETAILS 

beginning: 1999.

end: 2002.

Country of research: Italy

Counry of funding source: Italy

Funding organization: Telethon

Financing: PRIVATE FUNDERS – 185 924 €

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