A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease.

Björkqvist, M., Wild, E.J., Thiele, J., Silvestroni, A., Andre, R., Lahiri, N., Raibon, E., Lee, R.V., Benn, C.L., Soulet, D., Magnusson, A., Woodman, B., Landles, C., Pouladi, M.A., Hayden, M.R., Khalili-Shirazi, A., Lowdell, M.W., Brundin, P., Bates, G.P., Leavitt, B.R., Möller, T., Tabrizi, S.J.
Journal   J Exp Med.
Species  
Analytes Measured   GM-CSF , IL-10 , IL-12 Total , IL-4 , IL-5 , IL-6 , IL-8
Matrix Tested   Plasma
Year   2008
Volume   205
Page Numbers   1869-77
Application   Alzheimers
Abstract
Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by both neurological and systemic abnormalities. We examined the peripheral immune system and found widespread evidence of innate immune activation detectable in plasma throughout the course of HD. Interleukin 6 levels were increased in HD gene carriers with a mean of 16 years before the predicted onset of clinical symptoms. To our knowledge, this is the earliest plasma abnormality identified in HD. Monocytes from HD subjects expressed mutant huntingtin and were pathologically hyperactive in response to stimulation, suggesting that the mutant protein triggers a cell-autonomous immune activation. A similar pattern was seen in macrophages and microglia from HD mouse models, and the cerebrospinal fluid and striatum of HD patients exhibited abnormal immune activation, suggesting that immune dysfunction plays a role in brain pathology. Collectively, our data suggest parallel central nervous system and peripheral pathogenic pathways of immune activation in HD.

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Multiplex
U-PLEX NHP IL-4 Antibody Set
IL-4 | Non-human primate
U-PLEX Training Pack, 4-Assay
IL-1β, IL-6, IL-8, TNF-α | Human
Multiplex
U-PLEX Training Pack, 10-Assay
IL-1β, IL-6, IL-8, TNF-α | Human
Multiplex
U-PLEX Biomarker Group 1 (hu) Assays
CTACK, ENA-78, Eotaxin, Eotaxin-2, Eotaxin-3, EPO, FLT3L, Fractalkine, G-CSF, GM-CSF, GRO-α, I-309, IFN-α2a, IFN-β, IFN-γ, IL-1α, IL-1β, IL-1RA, IL-2, IL-2Rα, IL-3, IL-4, IL-5, IL-6, IL-7, IL-8, IL-9, IL-10, IL-12/IL-23p40, IL-12p70, IL-13, IL-15, IL-16, IL-17A, IL-17A/F, IL-17B, IL-17C, IL-17D, IL-17E/IL-25, IL-17F, IL-18, IL-21, IL-22, IL-23, IL-27, IL-29/IFN-λ1, IL-31, IL-33, IP-10, I-TAC, MCP-1, MCP-2, MCP-3, MCP-4, M-CSF, MDC, MIF, MIP-1α, MIP-1β, MIP-3α, MIP-3β, MIP-5, SDF-1α, TARC, TGF-β1, TGF-β2, TGF-β3, TNF-α, TNF-β, TPO, TRAIL, TSLP, VEGF-A, YKL-40 | Human
Multiplex
U-PLEX Biomarker Group 1 (NHP) Assays
CTACK, ENA-78, Eotaxin, Eotaxin-2, Eotaxin-3, FLT3L, Fractalkine, G-CSF, GM-CSF, GRO-α, I-309, IFN-α2a, IFN-γ, IL-1α, IL-1β, IL-1RA, IL-2, IL-4, IL-5, IL-6, IL-7, IL-8, IL-9, IL-10, IL-12/IL-23p40, IL-12p70, IL-13, IL-15, IL-16, IL-17A, IL-17A/F, IL-17B, IL-17C, IL-17D, IL-17F, IL-18, IL-22, IL-23, IP-10, I-TAC, MCP-1, MCP-2, MCP-3, MCP-4, M-CSF, MDC, MIF, MIP-1α, MIP-1β, MIP-3α, MIP-3β, MIP-5, SDF-1α, TARC, TGF-β1, TGF-β2, TGF-β3, TNF-α, TNF-β, TPO, TRAIL, VEGF-A, YKL-40 | Non-human primate
Multiplex
U-PLEX Chemokine Combo 1 (hu)
Eotaxin, Eotaxin-2, Eotaxin-3, IL-8, IP-10, MCP-1, MCP-2, MCP-3, MCP-4, MDC, MIP-1α, MIP-1β, TARC | Human
Multiplex
U-PLEX Chemokine Combo 1 (NHP)
Eotaxin, Eotaxin-3, IL-8, IP-10, MCP-1, MCP-4, MDC, MIP-1α, MIP-1β, TARC | Non-human primate
Multiplex
U-PLEX Custom Biomarker Group 1 (NHP) Assays
CTACK, ENA-78, Eotaxin, Eotaxin-2, Eotaxin-3, FLT3L, Fractalkine, G-CSF, GM-CSF, GRO-α, I-309, IFN-α2a, IFN-γ, IL-1α, IL-1β, IL-1RA, IL-2, IL-4, IL-5, IL-6, IL-7, IL-8, IL-9, IL-10, IL-12/IL-23p40, IL-12p70, IL-13, IL-15, IL-16, IL-17A, IL-17A/F, IL-17B, IL-17C, IL-17D, IL-17F, IL-18, IL-22, IL-23, IP-10, I-TAC, MCP-1, MCP-2, MCP-3, MCP-4, M-CSF, MDC, MIF, MIP-1α, MIP-1β, MIP-3α, MIP-3β, MIP-5, SDF-1α, TARC, TGF-β1, TGF-β2, TGF-β3, TNF-α, TNF-β, TPO, TRAIL, VEGF-A, YKL-40 | Non-human primate
Multiplex
U-PLEX Custom Biomarker Group 1 Human Assays
CTACK, ENA-78, Eotaxin, Eotaxin-2, Eotaxin-3, EPO, FLT3L, Fractalkine, G-CSF, GM-CSF, GRO-α, I-309, IFN-α2a, IFN-β, IFN-γ, IL-1α, IL-1β, IL-1RA, IL-2, IL-2Rα, IL-3, IL-4, IL-5, IL-6, IL-7, IL-8, IL-9, IL-10, IL-12/IL-23p40, IL-12p70, IL-13, IL-15, IL-16, IL-17A, IL-17A/F, IL-17B, IL-17C, IL-17D, IL-17E/IL-25, IL-17F, IL-18, IL-21, IL-22, IL-23, IL-27, IL-29/IFN-λ1, IL-31, IL-33, IP-10, I-TAC, MCP-1, MCP-2, MCP-3, MCP-4, M-CSF, MDC, MIF, MIP-1α, MIP-1β, MIP-3α, MIP-3β, MIP-5, SDF-1α, TARC, TGF-β1, TGF-β2, TGF-β3, TNF-α, TNF-β, TPO, TRAIL, TSLP, VEGF-A, YKL-40 | Human
Multiplex
U-PLEX Cytokine Combo 1 (hu)
GM-CSF, IL-1α, IL-5, IL-7, IL-12/IL-23p40, IL-15, IL-16, IL-17A, TNF-β, VEGF-A | Human
Multiplex
U-PLEX Cytokine Combo 1 (NHP)
GM-CSF, IL-1α, IL-5, IL-7, IL-12/IL-23p40, IL-15, IL-16, IL-17A, TNF-β, VEGF-A | Non-human primate
Multiplex
U-PLEX Human GM-CSF Assay
GM-CSF | Human
Singleplex
U-PLEX Human IL-4 Assay
IL-4 | Human
Singleplex
U-PLEX Human IL-5 Assay
IL-5 | Human
Singleplex
U-PLEX Human IL-6 Assay
IL-6 | Human
Singleplex
U-PLEX Human IL-8 Assay
IL-8 | Human
Singleplex
U-PLEX Human IL-10 Assay
IL-10 | Human
Singleplex
U-PLEX Mouse GM-CSF Assay
GM-CSF | Mouse
Singleplex
U-PLEX Mouse IL-4 Assay
IL-4 | Mouse
Singleplex
U-PLEX Mouse IL-5 Assay
IL-5 | Mouse
Singleplex
U-PLEX Mouse IL-6 Assay
IL-6 | Mouse
Singleplex
U-PLEX Mouse IL-10 Assay
IL-10 | Mouse
Singleplex
U-PLEX NHP GM-CSF Assay
GM-CSF | Non-human primate
Singleplex
U-PLEX NHP IL-5 Assay
IL-5 | Non-human primate
Singleplex
U-PLEX NHP IL-6 Assay
IL-6 | Non-human primate
Singleplex
U-PLEX NHP IL-8 Assay
IL-8 | Non-human primate
Singleplex
U-PLEX NHP IL-10 Assay
IL-10 | Non-human primate
Singleplex
U-PLEX Proinflam Combo 1 (hu)
IFN-γ, IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, IL-12p70, IL-13, TNF-α | Human
Multiplex
U-PLEX Proinflam Combo 1 (NHP)
IFN-γ, IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, IL-12/IL-23p40, TNF-α | Non-human primate
Multiplex
U-PLEX Proinflam Combo 2 (hu)
GM-CSF, IFN-γ, IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, IL-12p70 | Human
Multiplex
U-PLEX Proinflam Combo 3 (hu)
IFN-γ, IL-1β, IL-6, TNF-α | Human
Multiplex
U-PLEX Proinflam Combo 4 (hu)
IL-1β, IL-6, IL-8, TNF-α | Human
Multiplex
U-PLEX TH1/TH2 Combo (hu)
IFN-γ, IL-1β, IL-2, IL-4, IL-5, IL-8, IL-10, IL-12p70, IL-13, TNF-α | Human
Multiplex
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