Journal
JOURNAL OF MOLECULAR NEUROSCIENCE
Volume 45, Issue 3, Pages 574-582Publisher
HUMANA PRESS INC
DOI: 10.1007/s12031-011-9622-3
Keywords
Progranulin; Frontotemporal lobar dementia; Sortilin; Tumor necrosis factor receptor; TDP-43; Neuroinflammation
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Funding
- NIA NIH HHS [P50 AG023501-03, P50 AG023501, P01 AG019724-05, P01 AG019724] Funding Source: Medline
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Frontotemporal lobar dementia (FTLD) is the most common cause of dementia in patients younger than 60 years of age, and causes progressive neurodegeneration of the frontal and temporal lobes usually accompanied by devastating changes in language or behavior in affected individuals. Mutations in the progranulin (GRN) gene account for a significant fraction of familial FTLD, and in the vast majority of cases, these mutations lead to reduced expression of progranulin via nonsense-mediated mRNA decay. Progranulin is a secreted glycoprotein that regulates a diverse range of cellular functions including cell proliferation, cell migration, and inflammation. Recent fundamental discoveries about progranulin biology, including the findings that sortilin and tumor necrosis factor receptor (TNFR) are high affinity progranulin receptors, are beginning to shed light on the mechanism(s) by which progranulin deficiency causes FTLD. This review will explore how alterations in basic cellular functions due to PGRN deficiency, both intrinsic and extrinsic to neurons, might lead to the development of FTLD.
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