Recent studies demonstrated that mice with SOX2 deletion in oligodendroglial cells exhibit CNS hypomyelination and develop ataxia and motor coordination defects.6,35 The current finding suggests that SOX2 functional deficiency in astroglial lineage may also contributes to the brain anomalies and neurological impairments in SOX2-mutated children. Here, SOX2 is linked to cerebellar ataxia.