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J Biol Chem. 2012 Sep 21;287(39):32298-306. Epub 2012 Aug 2.

Progranulin: a proteolytically processed protein at the crossroads of inflammation and neurodegeneration.

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1
Department of Neuroscience, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA.

Abstract

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 receptors for progranulin, but not granulin peptides, have been reported. Revealing the cell surface receptors and the intracellular functions of granulins and progranulin is crucial for understanding their contributions to neurodegeneration.

PMID:
22859297
PMCID:
PMC3463300
DOI:
10.1074/jbc.R112.399170
[Indexed for MEDLINE]
Free PMC Article
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