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Article

Pluripotent Stem Cell Model of Nakajo-Nishimura Syndrome Untangles Proinflammatory Pathways Mediated by Oxidative Stress.

Stem cell reports - 5 Jun 2018

Honda-Ozaki Fumiko, Terashima Madoka, Niwa Akira, Saiki Norikazu, Kawasaki Yuri, Ito Haruna, Hotta Akitsu, Nagahashi Ayako, Igura Koichi, Asaka Isao, Li Hongmei Lisa, Yanagimachi Masakatsu, Furukawa Fukumi, Kanazawa Nobuo, Nakahata Tatsutoshi, Saito Megumu K

Abstract excerpt

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...

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Source

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Identifiers

PMID: 29731430
DOI: 10.1016/j.stemcr.2018.04.004
PMCID: PMC5989695

Genes

PSMB8

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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