Immunoproteasome subunit β5i promotes perifascicular muscle atrophy in dermatomyositis by upregulating RIG-I
Authors' conclusion
The immunoproteasome subunit β5i promotes perifascicular muscle atrophy in DM via RIG-I upregulation
Study context
- Population
- Patients with dermatomyositis (DM) (muscle samples); human skeletal muscle myoblasts (HSMMs) in vitropatients with DM
- Participants
- 16 patients
Methodological detailsin vitro, lentivirus overexpression in HSMMs
- In vitro
- Lentivirus overexpression in HSMMs
Main finding
As reported in the abstract
- β5i and RIG-I expression in DM muscle significantly increased and closely associated with muscle disease severity
- Marked colocalised expression of β5i and RIG-I in perifascicular myofibres
- β5i overexpression in HSMMs significantly upregulated RIG-I, MuRF1, type I IFN-related proteins (MxA, IFNβ) and NF-κB pathway-related proteins (pIκBα, pIRF3, pNF-κBp65)
- HSMM viability decreased significantly after β5i overexpression, partly recovered by β5i inhibitor (PR957)
- RIG-I activation by pppRNA upregulated IFNβ and MuRF1 and reduced HSMM viability
Original publication
Immunoproteasome subunit β5i promotes perifascicular muscle atrophy in dermatomyositis by upregulating RIG-I. RMD Open. Published 28 February 2023. doi:10.
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