Sh3bp2 Gain-Of-Function Mutation Ameliorates Lupus Phenotypes in B6.MRL-Fas^lpr Mice.

ABSTRACT: SH3 domain-binding protein 2 (SH3BP2) is an adaptor protein that is predominantly expressed in immune cells, and it regulates intracellular signaling. We had previously reported that a gain-of-function mutation in SH3BP2 exacerbates inflammation and bone loss in murine arthritis models. Here, we explored the involvement of SH3BP2 in a lupus model. Sh3bp2 gain-of-function (P416R knock-in; Sh3bp2^KI/+) mice and lupus-prone B6.MRL-Fas^lpr mice were crossed to yield double-mutant (Sh3bp2^KI/+Fas^lpr/lpr) mice. We monitored survival rates and proteinuria up to 48 weeks of age and assessed renal damage and serum anti-double-stranded DNA antibody levels. Additionally, we analyzed B and T cell subsets in lymphoid tissues by flow c

SUBMITTER: Nagasu A 

PROVIDER: S-EPMC6562867 | biostudies-literature | 2019 Apr

REPOSITORIES: biostudies-literature