In humans, BCL11B expression is maintained at high levels in normal adult striatum but significantly decreased in Huntington disease (HD) cells, because mutant Huntington striatal neurons is sensitive to over-expression of BCL11B[144], suggesting that sequestration and/or decreased BCL11B expression is responsible for the deregulation of striatal gene expression and the specificity of pathology that are observed in HD. Here, BCL11B is linked to Huntington disease.