Clearance of peripheral nerve misfolded mutant protein by infiltrated macrophages correlates with motor neuron disease progression.

ABSTRACT: Macrophages expressing C-C chemokine receptor type 2 (CCR2) infiltrate the central and peripheral neural tissues of amyotrophic lateral sclerosis (ALS) patients. To identify the functional role of CCR2⁺ macrophages in the pathomechanisms of ALS, we used an ALS animal model, mutant Cu/Zn superoxide dismutase 1^G93A (mSOD1)-transgenic (Tg) mice. To clarify the CCR2 function in the model, we generated SOD1^G93A/CCR2^{Red fluorescence protein (RFP)/Wild type (WT)}/CX3CR1^{Green fluorescence protein (GFP)/WT}-Tg mice, which heterozygously express CCR2-RFP and CX3CR1-GFP, and SOD1^G93A/CCR2^RFP/RFP-Tg mice, which lack CCR2 protein expression and present with a CCR2-deficient phenotype. In mSOD1-Tg mice, mSOD1 accumulated in the s

SUBMITTER: Shiraishi W 

PROVIDER: S-EPMC8360983 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature