Network analysis of the brain proteome of <i>GRN</i> knockout mice reveals pathogenic mechanisms shared in human frontotemporal dementia caused by <i>GRN</i> mutations

Authors: Thomas Kukar, Meixiang Huang, Erica S Modeste, Eric B Dammer, Christopher J Holler, Qiudong Deng, Georgia L Taylor, Paola Merino, Nicholas T Seyfried

Published: 2020-12-07

DOI: 10.1002/alz.047569

Source: Full article


Abstract

AbstractBackgroundMutations in the GRN gene, encoding the progranulin (PGRN) protein, are a common cause of Frontotemporal dementia (FTD) and induce haploinsufficiency of PGRN. PGRN is composed of 7.5 repeating domains called granulins (GRNs 1‐7). PGRN is processed into GRNs within the lysosome, where we hypothesize they are critical for lysosome homeostasis and function. However, it is unclear how decreased levels of lysosomal PGRN/GRNs cause FTD, Alzheimer’s disease, and related neurodegenerative disorders.MethodsWe performed quantitative proteomic analysis of whole‐brain tissue from wild‐type (Grn