Review
Cell Biology
Chunzhu Song, Kendal Broadie
Summary: Drosophila models of neurological disease, particularly the FXS model, have greatly contributed to our understanding of the pathogenic mechanisms and neurological phenotypes of fragile X syndrome, and have provided potential therapeutic targets.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(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.
Article
Genetics & Heredity
Maria Dolores De Donno, Antonietta Puricella, Simona D'Attis, Valeria Specchia, Maria Pia Bozzetti
Summary: Fragile X syndrome is a neuro-developmental disease that affects intellectual abilities and social interactions. The fruit fly, Drosophila melanogaster, is a reliable model to study the neuronal pathways associated with this syndrome. It has been found that the FMRP protein is crucial for the normal structure of neurons, correct synaptic differentiation, and synaptic connectivity during neuronal circuit development. This study demonstrates that FMRP is also required for transposon silencing in the brains of Drosophila mutants, suggesting a potential role of transposons in the pathogenesis of Fragile X syndrome and abnormal social behaviors.
Article
Multidisciplinary Sciences
Zeynep Okray, Pedro F. F. Jacob, Ciara Stern, Kieran Desmond, Nils Otto, Clifford B. B. Talbot, Paola Vargas-Gutierrez, Scott Waddell
Summary: Multisensory learning enhances memory performance in Drosophila, even for individual sensory cues. The neural mechanisms underlying the binding of sensory features during learning and the augmentation of memory expression are still unknown.
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
Neurosciences
Zevelou Koza, Mohamad Ayajuddin, Abhik Das, Rahul Chaurasia, Limamanen Phom, Sarat Chandra Yenisetti
Summary: Sexual dysfunction (SD) is a common non-motor symptom of Parkinson's disease (PD) that is often overlooked. The mechanism of SD in PD is not well understood, but studies have shown that dopamine (DA) in the hypothalamus plays a role in regulating sexual behavior. In this study, a Drosophila melanogaster model of sporadic SD in PD was developed and observed based on abnormalities in courtship behavior markers. The model exhibited SD without mobility defects, and there was no observable degeneration of dopaminergic neurons in the brain. This study sheds light on the molecular basis of SD in PD and provides an opportunity for screening potential therapeutic molecules.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Behavioral Sciences
Hiroyuki Nakagawa, Shiori Maehara, Kazuhiko Kume, Hiroto Ohta, Jun Tomita
Summary: Oct alpha 2R, an octopamine receptor, regulates locomotor activity, grooming behavior, and starvation-induced hyperactivity in Drosophila.
GENES BRAIN AND BEHAVIOR
(2022)
Article
Clinical Neurology
Tarjani Shukla, June Bryan de la Pena, John M. Perish, Jonathan E. Ploski, Craig R. Stumpf, Kevin R. Webster, Catherine A. Thorn, Zachary T. Campbell
Summary: Fragile X syndrome is the most common inherited source of intellectual disability in humans, caused by epigenetic silencing of the Fmr1 gene. The MNK inhibitor eFT508 shows potential in alleviating deficits associated with FXS by targeting downstream pathways. This study highlights the ability of eFT508 to improve various phenotypic abnormalities linked to FXS.
Article
Multidisciplinary Sciences
Melanie R. Florkowski, Jessica L. Yorzinski
Summary: Animals experience stress and show physiological and behavioral responses to cope with it. This study investigates the effects of activating the D2 dopamine receptor on coping behavior in wild sparrows, and finds that it increases their biting behavior towards inanimate objects, suggesting it as a mechanism for stress coping.
Article
Neurosciences
Patricia Cogram, Luis C. C. Fernandez-Beltran, Maria Jose Casarejos, Sonia Sanchez-Yepes, Eulalia Rodriguez-Martin, Alfonso Garcia-Rubia, Maria Jose Sanchez-Barrena, Carmen Gil, Ana Martinez, Alicia Mansilla
Summary: Fragile X syndrome (FXS) is a genetic disorder that causes intellectual disability and autism. In this study, the researchers found that inhibiting the NCS-1/Ric8a complex can restore abnormal behavior and dopamine metabolism in FXS mice.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Audiology & Speech-Language Pathology
Xiao-yu Song, Wan-fu Wu, Yu-bing Dai, Hai-wei Xu, Andrew Roman, Li Wang, Margaret Warner, Jan-Ake Gustafsson
Summary: Age-related hearing loss is the most common type of hearing impairment. This study reveals a previously unreported role of LXR,B in maintaining SGNs, and its loss contributes to age-related cochlea degeneration.
Article
Neurosciences
Rachel Michelle Sare, Abigail Lemons, Carolyn Beebe Smith
Summary: Many patients with FXS have sleep disturbances, and FXS mice also have reduced sleep duration. Sleep is important for brain development and chronic sleep restriction affects behavior. Increasing sleep duration may improve behavior in FXS.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Clinical Neurology
Mayako Yamazaki, Takatomo Arai, Junko Yarimizu, Mitsuyuki Matsumoto
Summary: This study suggests that ASP5736 may be a potential drug for treating FXS and demonstrates its efficacy in improving hyperactivity and sensory motor gating deficits in Fmr1-targeted transgenic rats.
INTERNATIONAL JOURNAL OF NEUROPSYCHOPHARMACOLOGY
(2022)
Article
Multidisciplinary Sciences
Dominic J. Vita, Cole J. Meier, Kendal Broadie
Summary: Glia play a crucial role in remodeling neural circuits during development. FMRP acts within neurons to activate glial insulin receptors, facilitating Draper- and Shrub-dependent neuronal clearance.
NATURE COMMUNICATIONS
(2021)
Article
Clinical Neurology
Melodie Proteau-Lemieux, Angelina Lacroix, Luc Galarneau, Francois Corbin, Jean-Francois Lepage, Artuela Caku
Summary: This study confirms the safety of metformin in normoglycemic patients with FXS and suggests its potential in modifying GABA-mediated inhibition, a hallmark of FXS pathophysiology.
PROGRESS IN NEURO-PSYCHOPHARMACOLOGY & BIOLOGICAL PSYCHIATRY
(2021)
Article
Genetics & Heredity
Yiqin Jiang, Elise Pitmon, Jack Berry, Fred W. Wolf, Zach McKenzie, Tim J. Lebestky
G3-GENES GENOMES GENETICS
(2016)
Review
Immunology
Ju Chen, Elise Pitmon, Kepeng Wang
SEMINARS IN IMMUNOLOGY
(2017)
Article
Multidisciplinary Sciences
Jason C. You, Gabriel S. Stephens, Chia-Hsuan Fu, Xiaohong Zhang, Yin Liu, Jeannie Chin
Article
Oncology
Ju Chen, Xiaoyang Ye, Elise Pitmon, Mengqian Lu, Jun Wan, Evan R. Jellison, Adam J. Adler, Anthony T. Vella, Kepeng Wang
JOURNAL FOR IMMUNOTHERAPY OF CANCER
(2019)
Article
Neurosciences
Gabriel S. Stephens, Chia-Hsuan Fu, Corey P. St Romain, Yi Zheng, Justin J. Botterill, Helen E. Scharfman, Yin Liu, Jeannie Chin
FRONTIERS IN NEUROSCIENCE
(2020)
Article
Immunology
Zhichao Fan, Elise Pitmon, Lai Wen, Jacqueline Miller, Erik Ehinger, Rana Herro, Wei Liu, Ju Chen, Zbigniew Mikulski, Douglas J. Conrad, Alex Marki, Marco Orecchioni, Puja Kumari, Yanfang Peipei Zhu, Paola M. Marcovecchio, Catherine C. Hedrick, Craig A. Hodges, Vijay A. Rathinam, Kepeng Wang, Klaus Ley
Summary: Cystic fibrosis (CF) is a genetic disease characterized by recurrent lung infections and multiorgan inflammation. Defective adhesion and activation of monocytes in CF patients may contribute to disease progression. Transplanting wild-type bone marrow can replace defective monocytes, improve survival, and reduce inflammation in CF mice. These findings suggest potential therapeutic approaches for CF patients.
JOURNAL OF IMMUNOLOGY
(2022)
