Article
Medicine, General & Internal
Klaudia Plinta, Andrzej Plewka, Krzysztof Pawlicki, Nikola Zmarzly, Magdalena Wojcik-Pedziwiatr, Marcin Rudzinski, Agnieszka Krzak-Kubica, Magdalena Doregowska-Stachera, Monika Rudzinska-Bar
Summary: The study explored the relationship between plasma BDNF concentration and HD severity, finding that BDNF levels were inversely correlated with different stages of HD, but were not related to motor symptoms or cognitive impairment.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Cardiac & Cardiovascular Systems
Kobina Essandoh, Arasakumar Subramani, Olivia A. Ferro, James P. Teuber, Sribharat Koripella, Matthew J. Brody
Summary: Production and release of natriuretic peptides by the stressed heart can reduce cardiac workload and be targeted for the treatment of heart failure. However, the mechanisms regulating cardiomyocyte exocytosis and natriuretic peptide release are still unclear. Our study reveals a novel pathway involving zDHHC9-mediated palmitoylation of Rab3gap1, which impairs exocytosis and limits atrial natriuretic peptide release.
JACC-BASIC TO TRANSLATIONAL SCIENCE
(2023)
Article
Cell Biology
Abdellatif Benraiss, John N. Mariani, Mikhail Osipovitch, Adam Cornwell, Martha S. Windrem, Carlos Benitez Villanueva, Devin Chandler-Militello, Steven A. Goldman
Summary: Glial pathology is a causal contributor to the dysfunction of striatal neurons in Huntington's disease (HD). Through analyzing gene expression changes in astrocytes and microglia from mouse and human models, commonalities in glial pathobiology across species and models were identified. The data suggest that there are cell-type-specific changes in gene expression in glia expressing truncated mHTT compared to those expressing full-length mHTT, while also highlighting a conserved set of dysregulated pathways in HD glia.
Article
Pharmacology & Pharmacy
Samar M. Shawki, Mohammed A. Saad, Rania M. Rahmo, Walaa Wadie, Hanan S. El-Abhar
Summary: Liraglutide shows neuroprotective potential in a rat model of Huntington's disease by modulating multiple molecular signaling pathways and reducing apoptosis and oxidative stress, demonstrating a neurotherapeutic effect.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Cell Biology
Zhike Zhou, Shanshan Zhong, Rongwei Zhang, Kexin Kang, Xiaoqian Zhang, Ying Xu, Chuansheng Zhao, Mei Zhao
Summary: This study aimed to determine the molecular functions of BDNF in HD by identifying DEGs and constructing co-expression modules. Functional enrichment analyses revealed significant enrichment of DEGs in several pathways, including cAMP, MAPK, and Ras signaling pathways. Overall, low BDNF expression may mediate HD pathogenesis through cAMP, MAPK, and Ras signaling pathways.
Article
Pharmacology & Pharmacy
Irene Soles-Tarres, Nuria Cabezas-Llobet, Benjamin Lefranc, Jerome Leprince, Jordi Alberch, David Vaudry, Xavier Xifro
Summary: This study found that PACAP can exert antiapoptotic and neurotrophic effects on neuronal cells in Huntington's disease (HD) through the PAC1R receptor. In a mouse model, intranasal administration of PACAP restored motor function and increased levels of PAC1R, CBP, and BDNF in the striatum.
FRONTIERS IN PHARMACOLOGY
(2022)
Article
Cell Biology
Masataka Kunii, Yuria Noguchi, Shin-ichiro Yoshimura, Satoshi Kanda, Tomohiko Iwano, Erda Avriyanti, Nur Atik, Takashi Sato, Ken Sato, Masaharu Ogawa, Akihiro Harada
Summary: In developing brain, the SNARE complex composed of SNAP23, VAMP8, and Syntaxin1B plays a crucial role in the formation of apical junctional complexes (AJCs) and radial glial cell (RGC) polarization. Its depletion leads to severe hypoplasia of the neocortex, absence of hippocampus or cerebellum, loss of RGC polarity, and alterations in proliferation, differentiation, and apoptosis. The localization of AJC protein N-cadherin by SNAREs is essential for AJC formation and RGC polarization during brain development.
JOURNAL OF CELL BIOLOGY
(2021)
Article
Clinical Neurology
Huajing You, Tengteng Wu, Gang Du, Yue Huang, Yixuan Zeng, Lishan Lin, Dingbang Chen, Chao Wu, Xunhua Li, Jean-marc Burgunder, Zhong Pei
Summary: This study aimed to investigate the role of blood GFAP in evaluating the clinical severity of HD. The concentrations of plasma NfL and GFAP were analyzed and compared with clinical severity assessments, suggesting that GFAP may be a sensitive biomarker for evaluating HD progression.
FRONTIERS IN NEUROLOGY
(2021)
Review
Biochemistry & Molecular Biology
Nour F. Al-Ghraiybah, Junwei Wang, Amer E. Alkhalifa, Andrew B. Roberts, Ruchika Raj, Euitaek Yang, Amal Kaddoumi
Summary: This review provides an overview of recent advances on the role of microglia and astrocytes in neuroinflammation and provides updates on neuroinflammation diagnostic markers and investigational therapeutics currently in clinical trials.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biology
Tarik Seref Onur, Andrew Laitman, He Zhao, Ryan Keyho, Hyemin Kim, Jennifer Wang, Megan Mair, Huilan Wang, Lifang Li, Alma Perez, Maria de Haro, Ying-Wooi Wan, Genevera Allen, Boxun Lu, Ismael Al-Ramahi, Zhandong Liu, Juan Botas
Summary: Research shows that glia cells play a crucial role in the formation and maintenance of synapses in neurodegenerative diseases. Lowering genes involved in synapse assembly can alleviate pathological and behavioral deficits. Conserved gene dysregulation is observed in patients with neurodegenerative diseases, fruit flies, and mice.
Review
Cell Biology
Ruth Monk, Bronwen Connor
Summary: Huntington's disease is a neurodegenerative disorder characterized by the progressive decline of motor, cognitive, and psychiatric functions. Cell reprogramming technologies offer a revolutionary approach for modeling and investigating neurological conditions, including HD, with the potential for new therapeutic targets and drug screening. This review discusses the use of cell reprogramming to study HD and recent advancements in the field that hold promise for future treatments.
Review
Biochemistry & Molecular Biology
Chaebin Kim, Ali Yousefian-Jazi, Seung-Hye Choi, Inyoung Chang, Junghee Lee, Hoon Ryu
Summary: Huntington's disease is a rare neurodegenerative disorder caused by an expansion of CAG trinucleotide repeat in the HTT gene. The disease is characterized by involuntary movements, cognitive and psychiatric dysfunctions, primarily affecting the striatum in the brain. Understanding the precise neuropathological mechanisms of HD could improve therapeutic approaches for its treatment.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Karen E. E. Anderson, Max Stevenson, Rency Varghese, Michaeline L. L. Hebron, Erin Koppel, Mara McCartin, Robin Kuprewicz, Sara Matar, Dalila Ferrante, Charbel Moussa
Summary: Nilotinib, a tyrosine kinase inhibitor, affects dopamine transmission and gene expression, but does not significantly improve behavioral and motor symptoms in HD patients. CSF miRNA sequencing reveals changes in specific genes associated with neurotransmitters in the basal ganglia.
Article
Biochemistry & Molecular Biology
Mostafa A. Rabie, Heba I. Ibrahim, Noha N. Nassar, Reham M. Atef
Summary: Huntington's disease (HD) is a neurodegenerative disease characterized by progressive impairment in motor, behavioral, and cognitive functions. This study investigated the protective effects of the adenosine A1 receptor agonist CHA in an HD-like rat model induced by 3-nitropropionic acid (3NP). The results showed that CHA attenuated neuronal death, improved cognitive and motor deficits, and reduced inflammatory and oxidative stress markers. These effects were associated with the activation of the PI3K/Akt/CREB/BDNF pathway and a decrease in astrocyte and microglia activation. Overall, CHA may have a protective effect against HD by modulating oxidative stress, excitotoxicity, and inflammatory pathways.
CHEMICO-BIOLOGICAL INTERACTIONS
(2023)
Review
Cell Biology
Reed Berlet, Dorothy Anne Galang Cabantan, Daniel Gonzales-Portillo, Cesar V. Borlongan
Summary: Stem cell therapy is a potential treatment for stroke, Parkinson's disease, and Huntington's disease. Enriched environment and exercise can enhance the reconstruction of neural circuitry in stem cell transplantation.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)