ArticleStem cell reports2018
Pluripotent Stem Cell Model of Nakajo-Nishimura Syndrome Untangles Proinflammatory Pathways Mediated by Oxidative Stress.
Article in Stem cell reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 38 citations in OpenAlex.
- The homozygous founder Psmb8 variant of Nakajo-Nishimura syndrome/proteasome-associated autoinflammatory syndrome causes panniculitis-associated lipoatrophy and a shortened lifespan in mice.Scientific reports · 2026Article
- Modeling antithymocyte globulin-induced microvasculopathy using human iPSC-derived vascularized liver organoids.Cell reports. Medicine · 2025Article
- Self-organization of sinusoidal vessels in pluripotent stem cell-derived human liver bud organoids.Nature biomedical engineering · 2025Article
- Cellular models in autoinflammatory disease research.Clinical & translational immunology · 2024Review
- Investigation of immune-related diseases using patient-derived induced pluripotent stem cells.Inflammation and regeneration · 2023Review
- Recapitulation of pro-inflammatory signature of monocytes with ACVR1A mutation using FOP patient-derived iPSCs.Orphanet journal of rare diseases · 2022Article
- Induced Pluripotent Stem Cell-Derived Monocytes/Macrophages in Autoinflammatory Diseases.Frontiers in immunology · 2022Review
- An integrated analysis of human myeloid cells identifies gaps in in vitro models of in vivo biology.Stem cell reports · 2021Article
- The role of interferons type I, II and III in myositis: A review.Brain pathology (Zurich, Switzerland) · 2021Review
- Pluripotent stem cell-based screening identifies CUDC-907 as an effective compound for restoring the in vitro phenotype of Nakajo-Nishimura syndrome.Stem cells translational medicine · 2021Article
- Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells.Children (Basel, Switzerland) · 2021Review
- Article
- The ubiquitin-proteasome system in regulation of the skeletal muscle homeostasis and atrophy: from basic science to disorders.The journal of physiological sciences : JPS · 2020Review
- Article
- Verification and rectification of cell type-specific splicing of a Seckel syndrome-associated ATR mutation using iPS cell model.Journal of human genetics · 2019Article
- Mass Spectrometry-based Absolute Quantification of 20S Proteasome Status for ControlledMolecular & cellular proteomics : MCP · 2019Article
- Induced pluripotent stem cells representing Nakajo-Nishimura syndrome.Inflammation and regeneration · 2019Review
Corrections and comments
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Authors and funding
16 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Nakajo-Nishimura syndrome (NNS) is an immunoproteasome-associated autoinflammatory disorder caused by a mutation of the PSMB8 gene. Although dysfunction of the immunoproteasome causes various cellular stresses attributed to the overproduction of inflammatory cytokines and chemokines in NNS, the underlying mechanisms of the autoinflammation are still largely unknown. To investigate and understand the mechanisms and signal pathways in NNS, we established a panel of isogenic pluripotent stem cell (PSC) lines with PSMB8 mutation. Activity of the immunoproteasome in PSMB8-mutant PSC-derived myeloid cell lines (MT-MLs) was reduced even without stimulation compared with non-mutant-MLs. In addition, MT-MLs showed an overproduction of inflammatory cytokines and chemokines, with elevated reactive oxygen species (ROS) and phosphorylated p38 MAPK levels. Treatment with p38 MAPK inhibitor and antioxidants decreased the abnormal production of cytokines and chemokines. The current PSC model revealed a specific ROS-mediated inflammatory pathway, providing a platform for the discovery of alternative therapeutic options for NNS and related immunoproteasome disorders.
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Registered trials
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