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Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia

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Yin, Fangfang, Dumont, Magali, Banerjee, Rebecca, Ma, Yao, Li, Huihong, Lin, Michael T., Beal, M. Flint, Nathan, Carl, Thomas, Bobby, Ding, Aihao·2010·The FASEB Journal

DOI 10.1096/fj.10-161471PMC PMC2992364

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Abstract

ABSTRACT Progranulin haploinsufficiency causes frontotemporal dementia with tau‐negative, ubiq‐uitin‐positive neuronal inclusion pathology. In this study, we showed that progranulin‐deficient mice displayed increased depression‐ and disinhibition‐like behavior, as well as deficits in social recognition from a relatively young age. These mice did not have any deficit in locomotion or exploration. Eighteen‐month‐old progranulin‐deficient mice demonstrated impaired spatial learning and memory in the Morris water maze. In addition to behavioral deficits, progranulin‐deficient mice showed a progressive development of neuropathology from 12 mo of age, including enhanced activation of microglia and astro‐cytes and ubiquitination and cytoplasmic accumulation of phosphorylated TDP‐43. Thus, progranulin deficiency induced FTD‐like behavioral and neuro‐pathological deficits. These mice may serve as an important tool for deciphering underlying mechanisms in frontotemporal dementia.—Yin, F., Du‐mont, M., Banerjee, R., Ma, Y., Li, H., Lin, M. T., Beal, M. F., Nathan, C., Thomas, B., Ding, A. Behavioral deficits and progressive neuropathology in progranulin‐deficient mice: a mouse model of frontotemporal dementia. FASEB J. 24, 4639–4647 (2010). www.fasebj.org

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Authors: Yin, F., Dumont, M., Banerjee, R., Ma, Y., Li, H., Lin, M. T., Beal, M. F., Nathan, C., Thomas, B., & Ding, A.
Affiliations: Cornell University (US)
Weill Cornell Medicine (US)
Chinese Academy of Medical Sciences & Peking Union Medical College (CN)
ORCID: Dumont, Magali
Banerjee, Rebecca
Ma, Yao
Li, Huihong
Nathan, Carl
Thomas, Bobby
Ding, Aihao
Venue: The FASEB Journal
Publisher: Wiley
Published: 2010-12
Volume: 24
Issue: 12
Pages: 4639-4647
ISSN: 0892-6638 (ISSN-L)
1530-6860
DOI: 10.1096/fj.10-161471
PMC: PMC2992364
OpenAlex: W2106248056
Cited by: 180
References: 30
Citations by year: 2026: 1 · 2025: 5 · 2024: 8 · 2023: 11 · 2022: 4 · 2021: 12 · 2020: 12 · 2019: 10 · 2018: 17 · 2017: 20 · 2016: 14 · 2015: 15 · 2014: 12 · 2013: 8 · 2012: 13
FWCI: 7.44
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: The FASEB Journal (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health (GM061710, AI030165)
National Institute on Aging (R01AG020729)
National Institute of General Medical Sciences (R01GM061710, GM061710)
National Institute of Allergy and Infectious Diseases (AI030165, R01AI030165)
National Institute of Neurological Disorders and Stroke (R01NS060885, NS060885, NS062165, R21NS062165)
Topics: Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Keywords: progranulin, frontotemporal dementia, haploinsufficiency, behavioral deficits, depression-like behavior, social recognition, spatial learning and memory, microglial activation, astrocyte activation, ubiquitination, TDP-43 phosphorylation, neuropathology, mouse model
MeSH terms: Progranulins, Granulins, Animals, Disease Models, Animal, DNA-Binding Proteins, Immunohistochemistry, Maze Learning, Intercellular Signaling Peptides and Proteins, Mice, Frontotemporal Dementia

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Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia

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