Modeling the autistic cell: iPSCs recapitulate developmental principles of syndromic and nonsyndromic ASD. (25th April 2016)

• Record Type: Journal Article
• Authors: Ben‐Reuven, Lihi; Reiner, Orly
• Other Names: MARTINEZ S. guestEditor.; NAKAMURA H. guestEditor.
• Is Part Of: Development growth and differentiation. Volume 58:Number 5(2016)
• Page Start: 481
• Page End: 491
• Publication Date: 2016-04-25
• Subjects: autism -- brain development -- disease model -- induced pluripotent stem cell -- autism spectrum disorder; Embryology -- Periodicals; Developmental biology -- Periodicals; Growth -- Periodicals; 574.3
• DOI: 10.1111/dgd.12280
• Languages: English
• ISSNs: 0012-1592

Abstract

Abstract : The opportunity to model autism spectrum disorders (ASD) through generation of patient‐derived induced pluripotent stem cells (iPSCs) is currently an emerging topic. Wide‐scale research of altered brain circuits in syndromic ASD, including Rett Syndrome, Fragile X Syndrome, Angelman's Syndrome and sporadic Schizophrenia, was made possible through animal models. However, possibly due to species differences, and to the possible contribution of epigenetics in the pathophysiology of these diseases, animal models fail to recapitulate many aspects of ASD. With the advent of iPSCs technology, 3D cultures of patient‐derived cells are being used to study complex neuronal phenotypes, including both syndromic and nonsyndromic ASD. Here, we review recent advances in using iPSCs to study various aspects of the ASD neuropathology, with emphasis on the efforts to create in vitro model systems for syndromic and nonsyndromic ASD. We summarize the main cellular activity phenotypes and aberrant genetic interaction networks that were found in iPSC‐derived neurons of syndromic and nonsyndromic autistic patients. Abstract : The opportunity to model Autism Spectrum Disorders (ASD) through generation of patient‐derived induced pluripotent stem cells (iPSCs) is currently an emerging topic. Here, we summarize the main cellular activity phenotypes and aberrant genetic interaction networks found in iPSC‐derived neurons of syndromic and nonsyndromic autistic patients.