We observed that besides higher mRNA expression of MMP9, MMP14 and TIMP4, protein expression of MMP2, 3, 9 and 14 and TIMP1, 2, 3 and 4 is abundantly present in cortical tubers of patients with TSC and that particularly glial cells, dysmorphic neurons and giant cells were strongly immunopositive for MMPs. Here, TIMP4 is linked to tuberous sclerosis.