Recently, mutations in the progranulin ( PGRN ) gene were found to cause familial and apparently sporadic frontotemporal lobe dementia (FTLD). Moreover, missense changes in PGRN were identified in patients with motor neuron degeneration, a condition that is related to FTLD. Most mutations identified in patients with FTLD until now have been null mutations. However, it remains unknown whether PGRN protein levels are reduced in the central nervous system from such patients. The effects of PGRN on neurons also remain to be established. We report that PGRN levels are reduced in the cerebrospinal fluid from FTLD patients carrying a PGRN mutation. We observe that PGRN and GRN E (one of the proteolytic fragments of PGRN) promote neuronal survival and enhance neurite outgrowth in cultured neurons. These results demonstrate that PGRN/GRN is a neurotrophic factor with activities that may be involved in the development of the nervous system and in neurodegeneration. Footnotes Abbreviations used in this paper: ALS, amyotrophic lateral sclerosis; AM, acetyoxymethyl; CSF, cerebrospinal fluid; FTLD, frontotemporal lobe dementia; GFAP, glial fibrillary acidic protein; GRN, granulin; NF-H, neurofilament heavy chain; PGRN, progranulin; SLPI, secreted leucocyte protease inhibitor. Submitted: 10 December 2007 Accepted: 7 March 2008
The Journal of Cell Biology – Rockefeller University Press
Published: Apr 7, 2008
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