Wednesday, November 3, 2010

Friedreich's Ataxia Induced Pluripotent Stem Cells Model Intergenerational GAA⋅TTC Triplet Repeat Instability.

Cell Stem Cell. 2010 Nov 5;7(5):631-7.

Ku S, Soragni E, Campau E, Thomas EA, Altun G, Laurent LC, Loring JF, Napierala M, Gottesfeld JM.

Department of Molecular Biology, The Scripps Research Institute, 10550 N. Torrey Pines Road, La Jolla, CA 92037, USA.

Keywords: Friedreich's ataxia (FRDA), GAA⋅TTC triplet repeat, frataxin, heterochromatin-mediated gene silencing, induced pluripotent stem cells (iPSCs), fibroblasts, repeat instability, repair enzyme MSH2, shRNA silencing of MSH2.