[HTML][HTML] Progranulin: a proteolytically processed protein at the crossroads of inflammation and neurodegeneration

B Cenik, CF Sephton, BK Cenik, J Herz, G Yu - Journal of Biological …, 2012 - ASBMB
GRN mutations cause frontotemporal lobar degeneration with TDP-43-positive inclusions.
The mechanism of pathogenesis is haploinsufficiency. Recently, homozygous GRN
mutations were detected in two patients with neuronal ceroid lipofuscinosis, a lysosomal
storage disease. It is unknown whether the pathogenesis of these two conditions is related.
Progranulin is cleaved into smaller peptides called granulins. Progranulin and granulins are
attributed with roles in cancer, inflammation, and neuronal physiology. Cell surface …