Lysosomal β-glucuronidase regulates Lyme and rheumatoid arthritis severity

J Clin Invest. 2014 Jan;124(1):311-20. doi: 10.1172/JCI72339. Epub 2013 Dec 16.

Abstract

Lyme disease, caused by the spirochete Borrelia burgdorferi, is the most prevalent arthropod-borne illness in the United States and remains a clinical and social challenge. The spectrum of disease severity among infected patients suggests that host genetics contribute to pathogenic outcomes, particularly in patients who develop arthritis. Using a forward genetics approach, we identified the lysosomal enzyme β-glucuronidase (GUSB), a member of a large family of coregulated lysosomal enzymes, as a key regulator of Lyme-associated arthritis severity. Severely arthritic C3H mice possessed a naturally occurring hypomorphic allele, Gusbh. C57BL/6 mice congenic for the C3H Gusb allele were prone to increased Lyme-associated arthritis severity. Radiation chimera experiments revealed that resident joint cells drive arthritis susceptibility. C3H mice expressing WT Gusb as a transgene were protected from severe Lyme arthritis. Importantly, the Gusbh allele also exacerbated disease in a serum transfer model of rheumatoid arthritis. A known GUSB function is the prevention of lysosomal accumulation of glycosaminoglycans (GAGs). Development of Lyme and rheumatoid arthritis in Gusbh-expressing mice was associated with heightened accumulation of GAGs in joint tissue. We propose that GUSB modulates arthritis pathogenesis by preventing accumulation of proinflammatory GAGs within inflamed joint tissue, a trait that may be shared by other lysosomal exoglycosidases.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Arthritis, Experimental / enzymology*
  • Arthritis, Experimental / microbiology
  • Arthritis, Experimental / pathology
  • Arthritis, Rheumatoid / enzymology*
  • Arthritis, Rheumatoid / pathology
  • Borrelia burgdorferi*
  • Cells, Cultured
  • Glucuronidase / genetics
  • Glucuronidase / metabolism*
  • Glycosaminoglycans / metabolism
  • Humans
  • Joints / pathology
  • Lyme Disease / enzymology*
  • Lyme Disease / microbiology
  • Lyme Disease / pathology
  • Mice
  • Mice, Inbred C3H
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Sequence Deletion
  • Severity of Illness Index

Substances

  • Glycosaminoglycans
  • Glucuronidase