In vitro systems to study inborn errors of immunity using human induced pluripotent stem cells
In the last two decades, the exponential progress in the field of genetics could reveal the genetic impact on the onset and progression of several diseases affecting the immune system. This knowledge has led to the discovery of more than 400 monogenic germline mutations, also known as “inborn errors...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-11-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.1024935/full |
| _version_ | 1828098292085424128 |
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| author | Eirini Nikolouli Janne Reichstein Gesine Hansen Gesine Hansen Gesine Hansen Nico Lachmann Nico Lachmann Nico Lachmann Nico Lachmann |
| author_facet | Eirini Nikolouli Janne Reichstein Gesine Hansen Gesine Hansen Gesine Hansen Nico Lachmann Nico Lachmann Nico Lachmann Nico Lachmann |
| author_sort | Eirini Nikolouli |
| collection | DOAJ |
| description | In the last two decades, the exponential progress in the field of genetics could reveal the genetic impact on the onset and progression of several diseases affecting the immune system. This knowledge has led to the discovery of more than 400 monogenic germline mutations, also known as “inborn errors of immunity (IEI)”. Given the rarity of various IEI and the clinical diversity as well as the limited available patients’ material, the continuous development of novel cell-based in vitro models to elucidate the cellular and molecular mechanisms involved in the pathogenesis of these diseases is imperative. Focusing on stem cell technologies, this review aims to provide an overview of the current available in vitro models used to study IEI and which could lay the foundation for new therapeutic approaches. We elaborate in particular on the use of induced pluripotent stem cell-based systems and their broad application in studying IEI by establishing also novel infection culture models. The review will critically discuss the current limitations or gaps in the field of stem cell technology as well as the future perspectives from the use of these cell culture systems. |
| first_indexed | 2024-04-11T08:00:31Z |
| format | Article |
| id | doaj.art-9674080f81284c9b936d6b68c72dff43 |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-11T08:00:31Z |
| publishDate | 2022-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-9674080f81284c9b936d6b68c72dff432022-12-22T04:35:45ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-11-011310.3389/fimmu.2022.10249351024935In vitro systems to study inborn errors of immunity using human induced pluripotent stem cellsEirini Nikolouli0Janne Reichstein1Gesine Hansen2Gesine Hansen3Gesine Hansen4Nico Lachmann5Nico Lachmann6Nico Lachmann7Nico Lachmann8Department for Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School (MHH), Hannover, GermanyDepartment for Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School (MHH), Hannover, GermanyDepartment for Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School (MHH), Hannover, GermanyCluster of Excellence - Resolving Infection Susceptibility (RESIST, EXC 2155), Hannover Medical School, Hannover, GermanyBiomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research (DZL), Hannover, GermanyDepartment for Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School (MHH), Hannover, GermanyCluster of Excellence - Resolving Infection Susceptibility (RESIST, EXC 2155), Hannover Medical School, Hannover, GermanyBiomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research (DZL), Hannover, GermanyRegenerative Biology to Reconstructive Therapy (REBIRTH) Center for Translational and Regenerative Medicine, Hannover Medical School, Hannover, GermanyIn the last two decades, the exponential progress in the field of genetics could reveal the genetic impact on the onset and progression of several diseases affecting the immune system. This knowledge has led to the discovery of more than 400 monogenic germline mutations, also known as “inborn errors of immunity (IEI)”. Given the rarity of various IEI and the clinical diversity as well as the limited available patients’ material, the continuous development of novel cell-based in vitro models to elucidate the cellular and molecular mechanisms involved in the pathogenesis of these diseases is imperative. Focusing on stem cell technologies, this review aims to provide an overview of the current available in vitro models used to study IEI and which could lay the foundation for new therapeutic approaches. We elaborate in particular on the use of induced pluripotent stem cell-based systems and their broad application in studying IEI by establishing also novel infection culture models. The review will critically discuss the current limitations or gaps in the field of stem cell technology as well as the future perspectives from the use of these cell culture systems.https://www.frontiersin.org/articles/10.3389/fimmu.2022.1024935/fullinborn errors of immunityiPSCsdisease modelingcell therapiesimmune cellsmacrophages |
| spellingShingle | Eirini Nikolouli Janne Reichstein Gesine Hansen Gesine Hansen Gesine Hansen Nico Lachmann Nico Lachmann Nico Lachmann Nico Lachmann In vitro systems to study inborn errors of immunity using human induced pluripotent stem cells Frontiers in Immunology inborn errors of immunity iPSCs disease modeling cell therapies immune cells macrophages |
| title | In vitro systems to study inborn errors of immunity using human induced pluripotent stem cells |
| title_full | In vitro systems to study inborn errors of immunity using human induced pluripotent stem cells |
| title_fullStr | In vitro systems to study inborn errors of immunity using human induced pluripotent stem cells |
| title_full_unstemmed | In vitro systems to study inborn errors of immunity using human induced pluripotent stem cells |
| title_short | In vitro systems to study inborn errors of immunity using human induced pluripotent stem cells |
| title_sort | in vitro systems to study inborn errors of immunity using human induced pluripotent stem cells |
| topic | inborn errors of immunity iPSCs disease modeling cell therapies immune cells macrophages |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.1024935/full |
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