Mucopolysaccharidosis IIIB, a lysosomal storage disease, triggers a pathogenic CNS autoimmune response.

Journal of Neuroinflammation
Smruti KilledarHaiyan Fu

Abstract

Recently, using a mouse model of mucopolysaccharidosis (MPS) IIIB, a lysosomal storage disease with severe neurological deterioration, we showed that MPS IIIB neuropathology is accompanied by a robust neuroinflammatory response of unknown consequence. This study was to assess whether MPS IIIB lymphocytes are pathogenic. Lymphocytes from MPS IIIB mice were adoptively transferred to naïve wild-type mice. The recipient animals were then evaluated for signs of disease and inflammation in the central nervous system. Our results show for the first time, that lymphocytes isolated from MPS IIIB mice caused a mild paralytic disease when they were injected systemically into naïve wild-type mice. This disease is characterized by mild tail and lower trunk weakness with delayed weight gain. The MPS IIIB lymphocytes also trigger neuroinflammation within the CNS of recipient mice characterized by an increase in transcripts of IL2, IL4, IL5, IL17, TNFalpha, IFNalpha and Ifi30, and intraparenchymal lymphocyte infiltration. Our data suggest that an autoimmune response directed at CNS components contributes to MPS IIIB neuropathology independent of lysosomal storage pathology. Adoptive transfer of purified T-cells will be needed in future studies...Continue Reading

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Citations

Apr 24, 2013·Journal of Inherited Metabolic Disease·N Joan Abbott
May 9, 2013·Journal of Inherited Metabolic Disease·Louise D ArcherJames E Fildes
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Jun 14, 2013·Journal of Child Neurology·Arushi Gahlot SainiMan Updesh Singh Sachdeva
Mar 2, 2017·International Journal of Molecular Sciences·Cátia S PereiraM Fatima Macedo
Feb 6, 2020·Journal of Clinical Medicine·Rachel Heon-RobertsAlexey V Pshezhetsky
Sep 19, 2020·Journal of Inherited Metabolic Disease·Berna Seker YilmazJulien Baruteau

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Methods Mentioned

BETA
PCR

Software Mentioned

BD FACSDiva

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