Glia in FTLD-GRN: from supporting cast to leading role.

Autor: Pinarbasi ES; Department of Pathology and., Barmada SJ; Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA.
Jazyk: angličtina
Zdroj: The Journal of clinical investigation [J Clin Invest] 2023 Mar 15; Vol. 133 (6). Date of Electronic Publication: 2023 Mar 15.
DOI: 10.1172/JCI168215
Abstrakt: A subset of the neurodegenerative disease frontotemporal lobar degeneration (FTLD) is caused by mutations in the progranulin (GRN) gene. In this issue of the JCI, Marsan and colleagues demonstrate disease-specific transcriptional profiles in multiple glial cell lineages - astrocytes, microglia, and oligodendroglia - that are highly conserved between patients with FTLD-GRN and the widely used Grn-/- mouse model. Additionally, the authors show that Grn-/- astrocytes fail to adequately maintain synapses in both mouse and human models. This study presents a compelling argument for a central role for glia in neurodegeneration and creates a rich resource for extending mechanistic insight into pathophysiology, identifying potential biomarkers, and developing therapeutic approaches.
Databáze: MEDLINE