MGI:1927086Mice homozygous for a disruption in this gene die by midgestation and exhibit cardiac development defects such as hemorrhage and cardiomyocyte apoptosis. Heterozygous mice exhibit axonal dystrophy in the nucleus gracilis, degeneration of Purkinje cells and gait abnormalities. mice homozygous for a conditional allele activated in neurons exhibit reduced NPC proliferation, reduced weight, abnormal dendrite arborization, seizures, and postnatal lethality.