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10.1016/j.ghir.2007.11.001 29 DichtelL

Snchez-Gonzlez, M.C., et al

The variant involved in C677T is a change from Cytosine (C) to Thymine (T) at position 677

Human ESC-derived chimeric mouse models of Huntingtons disease reveal cell-intrinsic defects in glial progenitor cell differentiation

This modification destabilized the protein in human cells but at the same time, increased its transactivation potential as judged from the luciferase assay results [55] (Figure 1B)