Article
Biochemistry & Molecular Biology
Lital Gildin, Rossana Rauti, Ofir Vardi, Liron Kuznitsov-Yanovsky, Ben M. Maoz, Menahem Segal, Dalit Ben-Yosef
Summary: Fragile X syndrome affects the development and function of human neuronal networks, with smaller cell bodies and reduced connections observed in FX-iNs compared to control iNs. FX networks also exhibit higher spontaneous burst-firing activity and lower network synchrony, providing insight into the intellectual dysfunction associated with FXS.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Cell Biology
Sebastien Gillotin, Vishal Sahni, Tjasa Lepko, Maya A. Hanspal, Jina E. Swartz, Zoi Alexopoulou, Fiona H. Marshall
Summary: Deficits in adult neurogenesis can lead to various neurological diseases, with recent studies exploring molecular mechanisms that could potentially lead to new therapeutic strategies. Understanding the role of adult neurogenesis is crucial for maintaining cognitive function and developing treatments for aging-related diseases, including neurodegeneration.
AGEING RESEARCH REVIEWS
(2021)
Article
Medicine, General & Internal
Sahar Javadi, Yue Li, Jie Sheng, Lucy Zhao, Yao Fu, Daifeng Wang, Xinyu Zhao
Summary: Transient Nutlin-3 treatment can prevent neurogenesis and cognitive deficits in mature adult FXS mice, potentially through modulating the adult neurogenic niche.
Review
Biochemistry & Molecular Biology
Daniela Valenti, Rosa Anna Vacca
Summary: Mitochondria play a crucial role in brain bioenergetics and cognitive function by integrating and controlling signaling pathways involved in neurogenesis and neuroplasticity. Dysfunctions in mitochondrial biogenesis and bioenergetics are associated with neurodevelopmental disorders, such as Down, Rett, and Fragile X syndromes. This review highlights the emerging role of mitochondria as master regulators of brain development and cognition, and discusses the potential of targeting mitochondria for therapeutic interventions.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Biochemistry & Molecular Biology
Pernille Bulow, Menahem Segal, Gary J. Bassell
Summary: This article discusses the emergence of hyperexcitability in neurodevelopmental disorders and explores recent advances in understanding novel mechanisms in Fragile X syndrome (FXS), providing new therapeutic strategies for FXS and other neurodevelopmental disorders.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Clinical Neurology
Ramkumar Aishworiya, Dragana Protic, Randi Hagerman
Summary: There is increasing recognition of the heterogeneity of origin of cases of autism spectrum disorder (ASD), with genetic etiology identified in 20-40% of cases. The Fragile X premutation state is a newly discovered disease state associated with various disorders, including ASD, and understanding molecular mechanisms may facilitate targeted treatments in the future.
JOURNAL OF NEUROLOGY
(2022)
Review
Biochemistry & Molecular Biology
Veronica Nobile, Cecilia Pucci, Pietro Chiurazzi, Giovanni Neri, Elisabetta Tabolacci
Summary: Fragile X syndrome (FXS) is the most common form of inherited causes of intellectual disability and autism, with no cure currently available. FXS is mainly caused by epigenetic inactivation of the FMR1 gene, but the molecular mechanisms responsible for gene silencing are not fully understood.
Article
Cell & Tissue Engineering
Ai Zhang, Irina Sokolova, Alain Domissy, Joshua Davis, Lee Rao, Kagistia Hana Utami, Yanling Wang, Randi J. Hagerman, Mahmoud A. Pouladi, Pietro Sanna, Michael J. Boland, Jeanne F. Loring
Summary: This study investigates the effects of Fragile X Syndrome (FXS) on GABAergic neurogenesis and suggests that loss of FMRP prolongs the proliferative stage of progenitors, leading to immature neurons in later stages of neurogenesis. This finding has implications for understanding the development of excitatory-inhibitory circuits in FXS and may guide therapeutic interventions.
STEM CELLS TRANSLATIONAL MEDICINE
(2022)
Article
Biochemistry & Molecular Biology
Katerina Skopalova, Katarzyna Anna Radaszkiewicz, Vera Kasparkova, Jaroslav Stejskal, Patrycja Bober, Ita Junkar, Miran Mozetic, Zdenka Capakova, Marian Lehocky, Martina Kasparova, Jiri Pachernik, Petr Humpolicek
Summary: Polypyrrole, as a representative of electro-conducting biomaterials, has been found to induce neurogenesis in embryonic bodies formed from embryonic stem cells through modulating signaling pathways. This effect is attributed to low-molecular-weight compounds present in aqueous polypyrrole extracts, leading to the importance of considering stem cell differentiation modulation when using polypyrrole as a biomaterial.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Cell Biology
Karo Talvio, Rimante Minkeviciene, Kayla G. Townsley, Venkat Swaroop Achuta, Laura M. Huckins, Padraic Corcoran, Kristen J. Brennand, Maija L. Castren
Summary: This study compared the transcriptomes of human neural progenitors (NPCs) generated from patient-derived induced pluripotent stem cells (iPSCs) of three fragile X syndrome (FXS) and three control male donors. The results showed altered expression of several genes related to triplet repeat instability, RNA splicing, testes development, and pathways previously shown to be affected in FXS. The study also revealed disturbed growth linked to reduced expression of LYNX1, suggesting dysregulated cholinergic system. The findings highlight the potential of human iPSCs in disease modeling and therapeutic interventions for FXS.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Article
Neurosciences
Nicole Edwards, Catharina Combrinck, Amy McCaughey-Chapman, Bronwen Connor
Summary: This study developed a novel approach to generate dorsal forebrain precursors from FXS patient-derived fibroblasts, leading to impaired neuronal development and maturation. Direct reprogramming offers a unique platform for further understanding the pathogenesis of FXS and identifying new drug targets for its treatment.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2023)
Article
Medicine, Research & Experimental
Mohaddeseh Sadat Alavi, Sajad Sahab Negah, Ahmad Ghorbani, Azar Hosseini, Hamid R. Sadeghnia
Summary: The study demonstrates that LEV can enhance the proliferation of rat embryonic neural stem cells and promote neuronal differentiation mainly through an NMDA receptor-mediated mechanism.
Article
Biochemistry & Molecular Biology
Rongzi Li, Wei Xiong, Boying Li, Yixuan Li, Bing Fang, Xifan Wang, Fazheng Ren
Summary: Adult hippocampal neurogenesis (AHN) is crucial for cognitive function and is dysregulated in Alzheimer's disease (AD). Plasmalogen (PLA), a group of phospholipids, has neuroprotective properties, but its effect on altered AHN in AD has not been studied. In this study, PLA was found to attenuate the decrease in neural stem cell viability and neuronal differentiation induced by β-amyloid (Aβ), partly through regulating the Wnt/β-catenin pathway. In AD mice, PLA supplementation improved impaired AHN and memory function. Therefore, PLA could regulate NSC differentiation and ameliorate AD-related memory impairment by up-regulating AHN.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Multidisciplinary Sciences
Ha Eun Kong, Junghwa Lim, Alexander Linsalata, Yunhee Kang, Indranil Malik, Emily G. Allen, Yiqu Cao, Lisa Shubeck, Rich Johnston, Yanting Huang, Yanghong Gu, Xiangxue Guo, Michael E. Zwick, Zhaohui Qin, Thomas S. Wingo, Jorge Juncos, David L. Nelson, Michael P. Epstein, David J. Cutler, Peter K. Todd, Stephanie L. Sherman, Stephen T. Warren, Peng Jin
Summary: This study identified Prosbeta5 (PSMB5) as a candidate genetic modifier for FXTAS using a Drosophila model. Knockdown of PSMB5 suppressed CGG-associated neurodegeneration in flies and cells. Additionally, an expression quantitative trait locus variant in PSMB5 was associated with delayed onset of FXTAS in human carriers. These findings suggest a therapeutic strategy for FXTAS by targeting PSMB5.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Biochemistry & Molecular Biology
Kaoru Tominaga, Eiji Sakashita, Katsumi Kasashima, Kenji Kuroiwa, Yasumitsu Nagao, Naoki Iwamori, Hitoshi Endo
Summary: Epigenetic factors play a crucial role in establishing functional organ systems during development, including brain development. However, the precise mechanism of epigenetic regulation in brain development and neurogenesis remains largely unknown. In this study, the researchers discovered the critical role of the Tip60 protein in brain development and the maintenance and function of neural stem/progenitor cells (NSCs). Tip60 deficiency led to multiple abnormalities in brain development, including microcephaly, reduced proliferation of developing brain cells, and impaired neural differentiation and migration. The study also revealed that Tip60 is involved in the transition from neurogenesis to gliogenesis during brain development.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Neurosciences
Michael Telias, Liron Kuznitsov-Yanovsky, Menahem Segal, Dalit Ben-Yosef
JOURNAL OF NEUROSCIENCE
(2015)
Editorial Material
Cell Biology
Michael Telias, Dalit Ben-Yosef
NEURAL REGENERATION RESEARCH
(2015)
Article
Neurosciences
Ivan Tochitsky, Zachary Helft, Victor Meseguer, Russell B. Fletcher, Kirstan A. Vessey, Michael Telias, Bristol Denlinger, Jonatan Malis, Erica L. Fletcher, Richard H. Kramer
Article
Neurosciences
Michael Telias, Menahem Segal, Dalit Ben-Yosef
FRONTIERS IN CELLULAR NEUROSCIENCE
(2016)
Article
Neurosciences
Michael Telias, Bristol Denlinger, Zachary Helft, Casey Thornton, Billie Beckwith-Cohen, Richard H. Kramer
Review
Neurosciences
Michael Telias
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2019)
Article
Cell & Tissue Engineering
Livia Preisler, Dalit Ben-Yosef, Yoav Mayshar
Review
Pharmacology & Pharmacy
Michael Telias
CURRENT PHARMACEUTICAL DESIGN
(2019)
Article
Biology
Noa Aharon-Hefetz, Idan Frumkin, Yoav Mayshar, Orna Dahan, Yitzhak Pilpel, Roni Rak
Article
Multidisciplinary Sciences
Livia Preisler, Aline Habib, Guy Shapira, Liron Kuznitsov-Yanovsky, Yoav Mayshar, Ilana Carmel-Gross, Mira Malcov, Foad Azem, Noam Shomron, Revital Kariv, Dov Hershkovitz, Dalit Ben-Yosef
Summary: The study found that embryonic stem cells with familial adenomatous polyposis (FAP) and different APC gene mutations may induce molecular defects leading to tumorigenic transformation, while genetic variations may also predict the severity of the disease.
SCIENTIFIC REPORTS
(2021)
Article
Biochemistry & Molecular Biology
Markus Mittnenzweig, Yoav Mayshar, Saifeng Cheng, Raz Ben-Yair, Ron Hadas, Yoach Rais, Elad Chomsky, Netta Reines, Anna Uzonyi, Lior Lumerman, Aviezer Lifshitz, Zohar Mukamel, Ayelet-Hashahar Orenbuch, Amos Tanay, Yonatan Stelzer
Summary: The mouse embryonic development model provides insights into mammalian cell fate acquisition. Recent studies show that most lineages exhibit combinatorial multifurcation dynamics rather than hierarchical transcriptional transitions.
Article
Multidisciplinary Sciences
Michael Telias, Kevin K. Sit, Daniel Frozenfar, Benjamin Smith, Arjit Misra, Michael J. Goard, Richard H. Kramer
Summary: Rod and cone photoreceptors degenerate in retinitis pigmentosa (RP). However, downstream neurons survive and undergo physiological changes. Retinoic acid (RA) is identified as the molecular trigger for hyperactivity in retinal ganglion cells (RGCs). Inhibiting RA synthesis with disulfiram improves visual perception in vision-impaired mice.
Article
Medicine, Research & Experimental
Bristol Denlinger, Zachary Helft, Michael Telias, Henri Lorach, Daniel Palanker, Richard H. Kramer