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American Journal of Alzheimer's Disease and Other Dementias®
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Article

Progranulin and B-Amyloid Distribution: A Case Report of the Brain From Preclinical PS-1 Mutation Carrier

Gediminas Gliebus, MD*, Andrea Rosso, MPH, and Carol F. Lippa, MD

* To whom correspondence should be addressed. E-mail: ggliebus{at}hotmail.com.


   Abstract
Background: Progranulin (PGRN) is a multifunctional growth factor that is found in many tissues. Mutations in the PGRN gene cause familial frontotemporal dementia with ubiquitin-positive inclusions. PGRN plaque-like structures have been described in Alzheimer's disease (AD), in association with {beta}-amyloid (A{beta}) plaques. Objective: To investigate PGRN and aggregated A{beta} immunolabeling distribution in autopsied brain tissue from the participant with confirmed PS-1 (A246E) mutation, who died prior to clinical symptom onset. Results: Immunolabeling for PGRN was positive and accumulated/formed plaque-like structures in all studied regions. These structures most frequently colocalized with A{beta} though there were some that did not. PGRN plaques were most dense in medial temporal and frontal regions and predominated over aggregated A{beta}. Conclusions: This case report illustrates PGRN accumulation and A{beta} aggregation in preclinical PS-1 AD case and raises the question whether this phenomenon coincides with or precedes A{beta} aggregation.

First published on September 23, 2009, doi:10.1177/1533317509346209

American Journal of Alzheimer's Disease and Other Dementias® 2009;24:456.

A more recent version of this article appeared on December 1, 2009


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