Increased neutrophil infiltration, IL-1 production and a SAPHO syndrome-like phenotype in PSTPIP2-deficient mice

Hsiu Jung Liao, I. Tsu Chyuan, Chien Sheng Wu, Shu Wha Lin, Kun Hung Chen, Huei-Fang Tsai, Ping Ning Hsu

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Objective. Proline-serine-threonine-phosphatase-interacting protein 2 (PSTPIP2) is involved in macrophage activation, neutrophil motility and osteoclast differentiation. However, the role of PSTPIP2 in inflammation and autoinflammatory diseases is still not clear. In this study, we generated PSTPIP2 knockout (Pstpip2-/-) mice to investigate its phenotype and role in autoinflammatory diseases. Methods. We constructed a Pstpip2-targeting vector and generated Pstpip2-/- mice. The phenotype and immunopathology of Pstpip2-/- mice were analysed. Results. All Pstpip2-/- mice developed paw swelling, synovitis, hyperostosis and osteitis, resembling SAPHO syndrome, an inflammatory disorder of the bone, skin and joints. Multifocal osteomyelitis was found in inflamed paws, with increased macrophage and marked neutrophil infiltrations in the bone, joint and skin. Profound osteolytic lesions with markedly decreased bone volume density developed in paws and limbs. Neutrophil-attracting chemokines and IL-1β were markedly elevated in inflamed tissues. Conclusion. Our study suggests that PSTPIP2 could play a role in innate immunity and development of autoinflammatory bone disorders, and may be associated with the pathogenesis of human SAPHO syndrome.

Original languageEnglish
Article numberkeu481
Pages (from-to)1317-1326
Number of pages10
JournalRheumatology
Volume54
Issue number7
DOIs
Publication statusPublished - Jul 1 2015

Keywords

  • IL-1
  • Neutrophil
  • PSTPIP2
  • SAPHO syndrome

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

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    Liao, H. J., Chyuan, I. T., Wu, C. S., Lin, S. W., Chen, K. H., Tsai, H-F., & Hsu, P. N. (2015). Increased neutrophil infiltration, IL-1 production and a SAPHO syndrome-like phenotype in PSTPIP2-deficient mice. Rheumatology, 54(7), 1317-1326. [keu481]. https://doi.org/10.1093/rheumatology/keu481