The Role of iPSC Modeling Toward Projection of Autophagy Pathway in Disease Pathogenesis: Leader or Follower.
Autophagy
Primary autophagy induced defects
Secondary autophagy induced defects
iPSC
Journal
Stem cell reviews and reports
ISSN: 2629-3277
Titre abrégé: Stem Cell Rev Rep
Pays: United States
ID NLM: 101752767
Informations de publication
Date de publication:
04 2021
04 2021
Historique:
accepted:
28
10
2020
pubmed:
28
11
2020
medline:
4
3
2022
entrez:
27
11
2020
Statut:
ppublish
Résumé
Autophagy is responsible for degradation of non-essential or damaged cellular constituents and damaged organelles. The autophagy pathway maintains efficient cellular metabolism and reduces cellular stress by removing additional and pathogenic components. Dysfunctional autophagy underlies several diseases. Thus, several research groups have worked toward elucidating key steps in this pathway. Autophagy can be studied by animal modeling, chemical modulators, and in vitro disease modeling with induced pluripotent stem cells (iPSC) as a loss-of-function platform. The introduction of iPSC technology, which has the capability to maintain the genetic background, has facilitated in vitro modeling of some diseases. Furthermore, iPSC technology can be used as a platform to study defective cellular and molecular pathways during development and unravel novel steps in signaling pathways of health and disease. Different studies have used iPSC technology to explore the role of autophagy in disease pathogenesis which could not have been addressed by animal modeling or chemical inducers/inhibitors. In this review, we discuss iPSC models of autophagy-associated disorders where the disease is caused due to mutations in autophagy-related genes. We classified this group as "primary autophagy induced defects (PAID)". There are iPSC models of diseases in which the primary cause is not dysfunctional autophagy, but autophagy is impaired secondary to disease phenotypes. We call this group "secondary autophagy induced defects (SAID)" and discuss them. Graphical abstract.
Identifiants
pubmed: 33245492
doi: 10.1007/s12015-020-10077-8
pii: 10.1007/s12015-020-10077-8
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
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