Trem2 Enhances Demyelination in the Csf1r^+/- Mouse Model of Leukoencephalopathy.

ABSTRACT: Colony-stimulating factor-1 receptor (CSF-1R)-related leukoencephalopathy (CRL) is a neurodegenerative disease that triggers early demyelination, leading to an adult-onset dementia. Triggering receptor expressed on myeloid cells-2 (TREM2) is a microglial receptor that promotes the activation of microglia and phagocytic clearance of apoptotic neurons and myelin debris. We investigated the role of Trem2 in the demyelination observed in the Csf1r^+/- mouse model of CRL. We show that elevation of Trem2 expression and callosal demyelination occur in 4-5-month-old Csf1r^+/- mice, prior to the development of symptoms. Absence of Trem2 in the Csf1r^+/- mouse attenuated myelin pathology and normalized microglial densities and morpholo

SUBMITTER: Biundo F 

PROVIDER: S-EPMC10452898 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature