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Researchers at University of British Columbia target dementia

Mental Health News Daily

2021 APR 20 (NewsRx) -- By a News Reporter-Staff News Editor at Mental Health News Daily -- A new study on dementia is now available. According to news originating from the University of British Columbia by NewsRx correspondents, research stated, “Frontotemporal lobar degeneration (FTLD) causes a spectrum of clinical presentations of frontotemporal dementia (FTD), including progressive changes in behavior, personality, executive function, and language.”

Funders for this research include Weston Brain Institute; Michael Smith Foundation for Health Research/Pacific Alzheimer Research Foundation.

Our news journalists obtained a quote from the research from University of British Columbia: “Up to 20% of familial FTLD cases are caused by progranulin (GRN) haploinsufficiency (FTD-GRN), with one of the most common causal variant being a nonsense mutation at arginine 493 (R493X). Recently, a genetic knockin FTD-GRN mouse model was generated bearing this Grn R493X mutation, at the analogous arginine in murine Grn. Aged, homozygous Grn R493X mice (Grn R493X/R493X ) have been shown to phenotypically replicate several neuropathological hallmarks previously demonstrated in Grn null mice. We conducted a comprehensive neuropathological and behavioral assessment of 18 month old Grn R493X/R493X mice, observing a striking lysosomal dysfunction and thalamic neurodegeneration not previously described in this model, as well as a male-specific increase in generalized anxiety.”

According to the news reporters, the research concluded: “These findings provide additional phenotypic markers of pathogenesis in aged Grn R493X/R493X mice that will contribute to better defining mechanisms underlying FTD-GRN, and offer relevant outcome measures for preclinical efficacy testing of novel therapeutics that target nonsense mutations leading to this devastating disease.”

For more information on this research see: Neuropathological and behavioral characterization of aged Grn R493X progranulin-deficient frontotemporal dementia knockin mice. Acta Neuropathologica Communications, 2021,9(1):1-16. (Acta Neuropathologica Communications - http://actaneurocomms.biomedcentral.com). The publisher for Acta Neuropathologica Communications is BMC.

A free version of this journal article is available at https://doi.org/10.1186/s40478-021-01158-x.

Our news editors report that additional information may be obtained by contacting Jonathan Frew, Djavad Mowafaghian Centre for Brain Health and Division of Neurology, University of British Columbia. Additional authors for this research include Haakon Berge Nygaard.

(Our reports deliver fact-based news of research and discoveries from around the world.)