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Progranulin is a highly conserved secreted protein that is expressed in multiple cell types, both in the CNS and in peripheral tissues. Both directly and via its conversion to granulins, progranulin regulates cell growth, survival, repair, and inflammation. Progranulin has a major role in regulation of lysosomal function and microglial responses in the CNS. Autosomal dominant mutations of the progranulin (GRN) gene leading to protein haploinsufficiency are linked to familial frontotemporal dementia with neuropathologic frontotemporal lobar degeneration (FTLD) associated with accumulation of TAR-DNA binding protein of 43kDA (TDP-43) inclusions (FTLD-TDP). Homozygous GRN mutations are linked to neuronal ceroid lipofuscinosis (NCL). These findings have stimulated interest in elucidating the normal regulation and function of progranulin and granulins and the pathomechanisms by which progranulin deficiency leads to neurodegeneration. These topics have been recently reviewed1–5 and some salient aspects are discussed here.
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- Copyright © 2017 American Academy of Neurology
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