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

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.

journal_name

Stem Cell Reports

journal_title

Stem cell reports

authors

Honda-Ozaki F,Terashima M,Niwa A,Saiki N,Kawasaki Y,Ito H,Hotta A,Nagahashi A,Igura K,Asaka I,Li HL,Yanagimachi M,Furukawa F,Kanazawa N,Nakahata T,Saito MK

doi

10.1016/j.stemcr.2018.04.004

subject

Has Abstract

pub_date

2018-06-05 00:00:00

pages

1835-1850

issue

6

issn

2213-6711

pii

S2213-6711(18)30172-3

journal_volume

10

pub_type

杂志文章

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