Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells

Autoinflammatory diseases are a disease entity caused by the dysregulation of innate immune cells. Typical autoinflammatory diseases are monogenic disorders and often very rare. As a result, there is a relative lack of understanding of the pathogenesis, poor diagnosis and little available treatment....

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Main Author: Megumu K. Saito
Format: Article
Language:English
Published: MDPI AG 2021-02-01
Series:Children
Subjects:
Online Access:https://www.mdpi.com/2227-9067/8/2/94
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author Megumu K. Saito
author_facet Megumu K. Saito
author_sort Megumu K. Saito
collection DOAJ
description Autoinflammatory diseases are a disease entity caused by the dysregulation of innate immune cells. Typical autoinflammatory diseases are monogenic disorders and often very rare. As a result, there is a relative lack of understanding of the pathogenesis, poor diagnosis and little available treatment. Induced pluripotent stem (iPS) cells are a new technology being applied to in vitro disease modeling. These models are especially useful for the analysis of rare and intractable diseases including autoinflammatory diseases. In this review, I will provide a general overview of iPS cell models for autoinflammatory diseases and a brief description of the results obtained from individual reports.
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spelling doaj.art-8916731872934e93907591bc468581682023-12-03T11:59:03ZengMDPI AGChildren2227-90672021-02-01829410.3390/children8020094Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS CellsMegumu K. Saito0Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University, Kyoto 6068507, JapanAutoinflammatory diseases are a disease entity caused by the dysregulation of innate immune cells. Typical autoinflammatory diseases are monogenic disorders and often very rare. As a result, there is a relative lack of understanding of the pathogenesis, poor diagnosis and little available treatment. Induced pluripotent stem (iPS) cells are a new technology being applied to in vitro disease modeling. These models are especially useful for the analysis of rare and intractable diseases including autoinflammatory diseases. In this review, I will provide a general overview of iPS cell models for autoinflammatory diseases and a brief description of the results obtained from individual reports.https://www.mdpi.com/2227-9067/8/2/94iPS cellsautoinflammatory diseasesmacrophagessomatic mosaicismgenome editing
spellingShingle Megumu K. Saito
Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
Children
iPS cells
autoinflammatory diseases
macrophages
somatic mosaicism
genome editing
title Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
title_full Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
title_fullStr Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
title_full_unstemmed Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
title_short Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
title_sort elucidation of the pathogenesis of autoinflammatory diseases using ips cells
topic iPS cells
autoinflammatory diseases
macrophages
somatic mosaicism
genome editing
url https://www.mdpi.com/2227-9067/8/2/94
work_keys_str_mv AT megumuksaito elucidationofthepathogenesisofautoinflammatorydiseasesusingipscells