Abstract
Myeloid-derived immune cells, including microglia, macrophages and monocytes, have been previously implicated in neurodegeneration. We investigated the role of infiltrating peripheral blood mononuclear cells (PBMC) in neuroinflammation and neurodegeneration in the HexB-/- mouse model of Sandhoff disease. Ablation of the chemokine receptor CCR2 in the HexB-/- mouse resulted in significant inhibition of PBMC infiltration into the brain, decrease in TNFα and MHC-II mRNA abundance and retardation in clinical disease development. There was no change in the level of GM2 storage and pro-apoptotic activity or astrocyte activation in HexB-/-;Ccr2-/- double knockout mice, which eventually succumbed secondary to GM2 gangliosidosis.
| Original language | English |
|---|---|
| Pages (from-to) | 50-57 |
| Number of pages | 8 |
| Journal | Journal of Neuroimmunology |
| Volume | 203 |
| Issue number | 1 |
| DOIs | |
| State | Published - Oct 15 2008 |
Keywords
- CCR2
- Chemokine receptors
- Microglia
- Neurodegeneration
- Neuroinflammation
- Peripheral blood
- Sandhoff disease
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