GFAP and infantile neuronal ceroid lipofuscinosis: Ablation of reactive astrocytes with upregulated GFAP and vimentin expression, or deletion of these proteins in knockout models have resulted in increased neurodegeneration and immune cell infiltration in models of spinal cord injury and infantile neuronal ceroid lipofuscinosis, respectively (Faulkner et al., 2004; Macauley et al., 2011), supporting a protective role for reactive astrocytes.