Article
Nutrition & Dietetics
Cara J. Westmark
Summary: The study found associations between the use of soy-based infant formula and worsened autistic behaviors in females and males with fragile X syndrome. It suggests the need for prospective evaluation of soy-based infant formula on disease comorbidities in fragile X syndrome, and supports further investigation into early gastrointestinal problems in the syndrome. The findings also indicate that premutation fragile X mothers should be encouraged to breastfeed, based on data from the Fragile X Syndrome Nutrition Study.
Review
Genetics & Heredity
Aadil Yousuf, Nadeem Ahmed, Abrar Qurashi
Summary: Fragile X-associated tremor/ataxia syndrome (FXTAS) and fragile X syndrome (FXS) are distinct disorders caused by abnormal expansion of CGG repeats. FXTAS is a neurodegenerative disorder characterized by gene hyperexpression, while FXS is a neurodevelopmental disorder characterized by gene silencing. Non-canonical DNA and RNA structures formed from CGG repeat expansions can disrupt cellular processes and have different effects in these two disorders.
FRONTIERS IN GENETICS
(2022)
Article
Biochemistry & Molecular Biology
Luisa Speranza, Kardelen Dalim Filiz, Sarah Goebel, Carla Perrone-Capano, Salvatore Pulcrano, Floriana Volpicelli, Anna Francesconi
Summary: Structural, functional, and molecular alterations in excitatory spines are common in neurodevelopmental disorders. The researchers developed a method using DiI and immunofluorescence to characterize spines in native brain tissue. They found that mutant mice with Fragile X Syndrome displayed dysgenesis of spines, including an overabundance of abnormally elongated thin spines and cup-shaped spines.
Article
Neurosciences
Kaleb Dee Miles, Caleb Andrew Doll
Summary: Developmental changes in ionic balance play a crucial role in neural circuit formation. The shift of GABAergic neurotransmission from depolarizing to hyperpolarizing output is induced by changes in Cl- gradients and is delayed in Fragile X syndrome (FXS) models. The absence of FMRP protein, which regulates chloride transporter expression, can significantly impact FXS phenotypes. This perspective summarizes the expression of Cl- transporters and discusses the imbalances in inhibitory neurotransmission in FXS, highlighting potential therapeutic strategies.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Immunology
Peifeng Guo, Xinyu Yang, Xiaomeng Guo, Huaien Yang, Jiao Pan, Yue Li
Summary: Fragile X syndrome (FXS) is a common inherited intellectual disability caused by a lack of FMRP. Gastrointestinal problems in FXS are associated with gut microbial dysbiosis, inflammation, and increased intestinal permeability. Our findings demonstrate that omega-3 PUFAs improve autistic behaviors and gut homeostasis in FMRP-deficient mice by suppressing gut microbiota dysbiosis, offering a novel therapeutic approach for juvenile FXS treatment.
BRAIN BEHAVIOR AND IMMUNITY
(2023)
Review
Cell Biology
Rob Willemsen, R. Frank Kooy
Summary: Fragile X-related disorders are caused by expanded CGG repeats in the FMR1 gene and can manifest as either neurodegenerative or neurodevelopmental disorders. Mouse models have provided valuable insights into these disorders and their translational value for developing targeted therapies for intellectual disability and autism disorders.
DISEASE MODELS & MECHANISMS
(2023)
Article
Clinical Neurology
Jennifer M. Walters, Eung Chang Kim, Jiaren Zhang, Han Gil Jeong, Archit Bajaj, Brian C. Baculis, Gregory C. Tracy, Baher Ibrahim, Catherine A. Christian-Hinman, Daniel A. Llano, Graham R. Huesmann, Hee Jung Chung
Summary: This study is the first to demonstrate that the drug TC-2153 has inhibitory effects on hippocampal excitability and seizure severity in both sexes. TC-2153 can reduce seizure severity and decrease overall hippocampal excitability.
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
Neurosciences
Nicole K. Morrill, Aurelie Joly-Amado, Qingyou Li, Sahana Prabhudeva, Edwin J. Weeber, Kevin R. Nash
Summary: This study found that the reduction in Reelin may be related to FXS, and enhancing the Reelin signaling successfully rescued cognitive deficits in FXS mice, providing a feasible therapeutic approach.
EXPERIMENTAL NEUROLOGY
(2022)
Article
Neurosciences
Se Jin Jeon, Huiyoung Kwon, Ho Jung Bae, Edson Luck Gonzales, Junhyeong Kim, Hye Jin Chung, Dong Hyun Kim, Jong Hoon Ryu, Chan Young Shin
Summary: This study found that agmatine can reverse FXS symptoms in Fmr1 KO mouse model, including compulsions, learning and memory deficits, hyperactivity, aberrant social interaction, and communication deficit, while normalizing abnormal long-term potentiation and depression in the hippocampus.
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
Neurosciences
Snow Bach, Stephen Shovlin, Michael Moriarty, Barbara Bardoni, Daniela Tropea
Summary: RTT and FXS are two monogenetic neurodevelopmental disorders with overlapping features possibly due to interactions between MeCP2 and FMRP. Both syndromes affect brain development and result in dysregulation of common molecular signaling pathways.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2021)
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
Azalea Lee, Jie Xu, Zhexing Wen, Peng Jin
Summary: Fragile X syndrome is the most common inherited cause of intellectual disability and autism spectrum disorder. Human induced pluripotent stem cells provide a unique means of studying FXS pathophysiology specific to humans.
Article
Biochemistry & Molecular Biology
Dragana D. Protic, Ramkumar Aishworiya, Maria Jimena Salcedo-Arellano, Si Jie Tang, Jelena Milisavljevic, Filip Mitrovic, Randi J. Hagerman, Dejan B. Budimirovic
Summary: FXS is a neurodevelopmental disorder that can be improved through early diagnosis and interventions targeting behavior symptoms.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Behavioral Sciences
Emilie M. Sorensen, Freja Bertelsen, Pia Weikop, Maria M. Skovborg, Tue Banke, Kim R. Drasbek, Jorgen Scheel-Kruger
BEHAVIOURAL PHARMACOLOGY
(2015)
Article
Plant Sciences
Manavi Chatterjee, Rajkumar Verma, Reena Kumari, Seema Singh, Anil Kumar Verma, Anil Kumar Dwivedi, Gautam Palit
PHARMACEUTICAL BIOLOGY
(2015)
Article
Neurosciences
Anna Karina Hugger Toft, Camilla Johanne Lundbye, Tue G. Banke
JOURNAL OF NEUROSCIENCE
(2016)
Article
Physiology
Tue G. Banke
PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY
(2016)
Article
Multidisciplinary Sciences
Matthew B. Johnson, Xingshen Sun, Andrew Kodani, Rebeca Borges-Monroy, Kelly M. Girskis, Steven C. Ryu, Peter P. Wang, Komal Patel, Dilenny M. Gonzalez, Yu Mi Woo, Ziying Yan, Bo Liang, Richard S. Smith, Manavi Chatterjee, Daniel Coman, Xenophon Papademetris, Lawrence H. Staib, Fahmeed Hyder, Joseph B. Mandeville, P. Ellen Grant, Kiho Im, Hojoong Kwak, John F. Engelhardt, Christopher A. Walsh, Byoung-Il Bae
Article
Chemistry, Medicinal
Tyler D. Baguley, Hai-Chao Xu, Manavi Chatterjee, Angus C. Nairn, Paul J. Lombroso, Jonathan A. Ellman
JOURNAL OF MEDICINAL CHEMISTRY
(2013)
Correction
Chemistry, Medicinal
Tyler D. Baguley, Hai-Chao Xu, Manavi Chatterjee, Angus C. Nairn, Paul J. Lombroso, Jonathan A. Ellman
JOURNAL OF MEDICINAL CHEMISTRY
(2014)
Article
Neurosciences
Camilla J. Lundbye, Anna Karina H. Toft, Tue G. Banke
JOURNAL OF PHYSIOLOGY-LONDON
(2018)
Article
Biochemistry & Molecular Biology
Jian Xu, Manavi Chatterjee, Tyler D. Baguley, Jonathan Brouillette, Pradeep Kurup, Debolina Ghosh, Jean Kanyo, Yang Zhang, Kathleen Seyb, Chimezie Ononenyi, Ethan Foscue, George M. Anderson, Jodi Gresack, Gregory D. Cuny, Marcie A. Glicksman, Paul Greengard, TuKiet T. Lam, Lutz Tautz, Angus C. Nairn, Jonathan A. Ellman, Paul J. Lombroso
Article
Chemistry, Medicinal
Neelima Sinha, Navnath P. Karche, Mahip Kalyan Verma, Sameer S. Walunj, Prashant B. Nigade, Gourhari Jana, Sanjay P. Kurhade, Anil K. Hajare, Ajay R. Tilekar, Ganesh R. Jadhav, Baban R. Thube, Javed S. Shaikh, Sudhakar Balgude, Lairikyengbam Bikramjit Singh, Vijaya Mahimane, Shridhar K. Adurkar, Girish Hatnapure, Firoj Raje, Yogesh Bhosale, Dnyaneshwar Bhanage, Sachchidanand Sachchidanand, Ruchi Dixit, Rajesh Gupta, Anand M. Bokare, Manoj Dandekar, Ashish Bharne, Manavi Chatterjee, Sagar Desai, Sarita Koul, Dipak Modi, Maneesh Mehta, Vinod Patil, Minakshi Singh, Jayasagar Gundu, Rajan N. Goel, Chirag Shah, Sharad Sharma, Dhananjay Bakhle, Rajender Kumar Kamboj, Venkata P. Palle
JOURNAL OF MEDICINAL CHEMISTRY
(2020)
Article
Behavioral Sciences
Manavi Chatterjee, Priya Singh, Jian Xu, Paul J. Lombroso, Pradeep K. Kurup
BEHAVIOURAL BRAIN RESEARCH
(2020)
Article
Neurosciences
Manavi Chatterjee, Jeemin Kwon, Jessie Benedict, Marija Kamceva, Pradeep Kurup, Paul J. Lombroso
Summary: Loss of dendritic spines and decline in cognitive function are characteristics of Alzheimer's disease patients. Studies have shown that targeting the protein tyrosine phosphatase STEP can improve cognitive function and synaptic connectivity in models of AD, suggesting the potential of STEP inhibitors as therapeutic agents.
EXPERIMENTAL BRAIN RESEARCH
(2021)
Article
Psychiatry
Manavi Chatterjee, Rajkumar Verma, Vijai Lakshmi, Shibani Sengupta, Anil Kumar Verma, Abbas Ali Mahdi, Gautam Palit
ASIAN JOURNAL OF PSYCHIATRY
(2013)
Correction
Neurosciences
Lucia Privitera, Ellen L. Hogg, Matthias Gaestel, Mark J. Wall, Sonia A. L. Correa
Article
Neurosciences
Li-Ya Jiang, Guan-Hao Wang, Jing-Jiao Xu, Xiao-Li Li, Xiao-Yan Lin, Xiang Fang, Hong-Xu Zhang, Mei Feng, Chun-Ming Jiang
Summary: This study reveals the importance of LINC00473 in regulating temozolomide (TMZ) resistance in glioblastoma (GB) and its potential mechanism. By regulating the expression of CEBP alpha and MGMT, LINC00473 promotes the formation of chemoresistance. Furthermore, LINC00473 can transfer chemoresistance to adjacent sensitive cells through exosomes.
Article
Neurosciences
Olga Kopach, Tetyana Pivneva, Nataliya Fedirko, Nana Voitenko
Summary: This study found that diabetic animals exhibit severe xerostomia characterized by reduced saliva flow rate, diminished total protein content, and decreased amylase activity. The impaired saliva production in diabetes is associated with reduced and delayed intracellular Ca2+ signals in submandibular acinar cells, caused by malfunctioning mitochondria. Targeting malfunctioning mitochondria may be a potential strategy for the treatment of diabetic xerostomia.
Article
Neurosciences
Nicholas M. Timme, Cherish E. Ardinger, Seth D. C. Weir, Rachel Zelaya-Escobar, Rachel Kruger, Christopher C. Lapish
Summary: This study aimed to assess aversion-resistant drinking behavior in head-fixed mice and explore the relationship between non-consummatory behaviors and aversion-resistant drinking. The results showed that head-fixed mice exhibited heterogenous levels of aversion-resistant drinking and non-consummatory behaviors were related to the intensity of this behavior.
Article
Neurosciences
David R. Maguire, Charles P. France
Summary: Methocinnamox (MCAM) is a novel, long-acting opioid receptor antagonist that effectively decreases fentanyl self-administration and prevents opioid overdose in monkeys. The study demonstrates the potential therapeutic utility of MCAM in the treatment of opioid use disorder.
Article
Neurosciences
Xiang Li, Dan Feng, Shenglu Ma, Mingxing Li, Shulei Zhao, Man Tang
Summary: This study investigated the effects of fluoxetine on neurochemical, neurobiological, and neurobehavioral changes in different subregions of the hippocampus. The results showed that fluoxetine increased dialysate 5-HT, decreased membrane 5-HTT protein, and increased cytoplasmic fraction. Additionally, fluoxetine reduced immobility times in behavioral tests, with greater effects observed in the ventral subregion compared to the dorsal subregion.
Article
Neurosciences
Alexander V. Zholos, Mariia I. Melnyk, Dariia O. Dryn
Summary: Acetylcholine is an important neurotransmitter in visceral smooth muscles, activating M2 and M3 muscarinic receptors to cause smooth muscle excitation and contraction. This review focuses on the cellular and molecular mechanisms underlying acetylcholine-induced depolarisation and smooth muscle contraction, as well as the effects of anticholinergic drugs on gastrointestinal motility. The knowledge gained from recent studies has greatly expanded our understanding of these processes.
Article
Neurosciences
Zhenlong Li, Hsien-Yu Peng, Chau-Shoun Lee, Tzer-Bin Lin, Ming-Chun Hsieh, Cheng-Yuan Lai, Han-Fang Wu, Lih-Chyang Chen, Mei-Ci Chen, Dylan Chou
Summary: Methylone shows significant efficacy in treating depression and social deficits, making it an ideal candidate for anti-depressant medication.
Article
Neurosciences
Aline Freyssin, Allison Carles, Sarra Guehairia, Gilles Rubinstenn, Tangui Maurice
Summary: This study explores the potential of combining FENM and S1R agonists in the treatment of Alzheimer's disease. The results showed that most FENM-based combinations can protect against learning deficits caused by A beta 25-35, with better efficacy in short-term memory.
Article
Neurosciences
J. D. Lorente, J. Cuitavi, L. Rullo, S. Candeletti, P. Romualdi, L. Hipolito
Summary: This study analyzed the effects of pain on negative affect in different sexes and time courses, as well as the involvement of the dynorphinergic and corticotropin releasing factor systems in these pain-related behaviors. The results showed sex and time-dependent anxiety- and anhedonia-like behaviors induced by pain in female rats. The recruitment of KOR/DYN in the NAc was identified as a key neurological substrate mediating pain-induced behavioral alterations.
Article
Neurosciences
Rongjun Liu, Daofan Sun, Xiuzhong Xing, Qingge Chen, Bo Lu, Bo Meng, Hui Yuan, Lan Mo, Liufang Sheng, Jinwei Zheng, Qiusheng Wang, Junping Chen, Xiaowei Chen
Summary: The coexistence of pain and depression is frequently observed in patients with chronic pain and depression. Oxytocin, a neuropeptide, has been reported to relieve chronic pain and depressive symptoms. This study investigated the effect of intranasal oxytocin on neuropathic pain and comorbid depressive symptoms, and found that oxytocin attenuated depression-like behavior but did not alleviate mechanical hyperalgesia. The results suggest that intranasal oxytocin may have the potential to treat depressive symptoms in neuropathic pain patients.