All Relations between cerebellum purkinje cell and Ataxia

Publication Sentence Publish Date Extraction Date Species
Luis E Salazar Leon, Amanda M Brown, Heet Kaku, Roy V Sillito. Purkinje cell dysfunction causes disrupted sleep in ataxic mice. bioRxiv : the preprint server for biology. 2023-07-18. PMID:37461479. here, we used an ataxia mouse model generated by silencing purkinje cell neurotransmission ( 2023-07-18 2023-08-14 mouse
Henry G S Martin, Dimitri M Kullman. Basket to Purkinje Cell Inhibitory Ephaptic Coupling Is Abolished in Episodic Ataxia Type 1. Cells. vol 12. issue 10. 2023-07-06. PMID:37408217. basket to purkinje cell inhibitory ephaptic coupling is abolished in episodic ataxia type 1. 2023-07-06 2023-08-14 Not clear
Haoran Huang, Vikram G Shakkotta. Targeting Ion Channels and Purkinje Neuron Intrinsic Membrane Excitability as a Therapeutic Strategy for Cerebellar Ataxia. Life (Basel, Switzerland). vol 13. issue 6. 2023-06-28. PMID:37374132. in this review, we propose that perturbations in cerebellar purkinje neuron intrinsic membrane excitability, a result of ion channel dysregulation, is a common pathophysiologic mechanism that drives motor impairment and vulnerability to degeneration in cerebellar ataxias of widely differing genetic etiologies. 2023-06-28 2023-08-14 Not clear
Enza Ferrero, Eleonora Di Gregorio, Marta Ferrero, Erika Ortolan, Young-Ah Moon, Antonella Di Campli, Lisa Pavinato, Cecilia Mancini, Debasmita Tripathy, Marta Manes, Eriola Hoxha, Chiara Costanzi, Elisa Pozzi, Matteo Rossi Sebastiano, Nico Mitro, Filippo Tempia, Donatella Caruso, Barbara Borroni, Manuela Basso, Michele Sallese, Alfredo Brusc. Spinocerebellar ataxia 38: structure-function analysis shows ELOVL5 G230V is proteotoxic, conformationally altered and a mutational hotspot. Human genetics. 2023-05-18. PMID:37199746. a missense variant (c.689g>t p.gly230val) in elovl5 causes spinocerebellar ataxia subtype 38 (sca38), a neurodegenerative disorder characterized by autosomal dominant inheritance, cerebellar purkinje cell demise and adult-onset ataxia. 2023-05-18 2023-08-14 mouse
Hoda Ranjbar, Monavareh Soti, Kristi A Kohlmeier, Mahyar Janahmadi, Mohammad Shaban. Pharmacologic antagonism of CB1 receptors improves electrophysiological alterations in Purkinje cells exposed to 3-AP. BMC neuroscience. vol 24. issue 1. 2023-03-03. PMID:36869289. although ataxia is associated with cerebellar dysfunction, little is known about the effects of 3-ap exposure on purkinje cell electrophysiological properties. 2023-03-03 2023-08-14 Not clear
Fabrice Faure, Lidia Yshii, Toufic Renno, Isabelle Coste, Bastien Joubert, Virginie Desestret, Roland Liblau, Jérôme Honnora. A Pilot Study to Develop Paraneoplastic Cerebellar Degeneration Mouse Model. Cerebellum (London, England). 2023-02-02. PMID:36729270. ataxia and gait instability were assessed in treated mice as well as autoantibody levels, purkinje cell density, and immune infiltration in the cerebellum. 2023-02-02 2023-08-14 mouse
Karla P Figueroa, Collin J Anderson, Sharan Paul, Warunee Dansithong, Mandi Gandelman, Daniel R Scoles, Stefan M Puls. Slc9a6 mutation causes Purkinje cell loss and ataxia in the shaker rat. Human molecular genetics. 2023-01-09. PMID:36621975. slc9a6 mutation causes purkinje cell loss and ataxia in the shaker rat. 2023-01-09 2023-08-14 mouse
Karla P Figueroa, Collin J Anderson, Sharan Paul, Warunee Dansithong, Mandi Gandelman, Daniel R Scoles, Stefan M Puls. Slc9a6 mutation causes Purkinje cell loss and ataxia in the shaker rat. Human molecular genetics. 2023-01-09. PMID:36621975. the shaker rat carries a naturally occurring mutation leading to progressive ataxia characterized by purkinje cell (pc) loss. 2023-01-09 2023-08-14 mouse
Sylvie Picker-Minh, Ilaria Luperi, Ethiraj Ravindran, Nadine Kraemer, Sami Zaqout, Gisela Stoltenburg-Didinger, Olaf Ninnemann, Luis R Hernandez-Miranda, Shyamala Mani, Angela M Kaind. PTRH2 is Necessary for Purkinje Cell Differentiation and Survival and its Loss Recapitulates Progressive Cerebellar Atrophy and Ataxia Seen in IMNEPD Patients. Cerebellum (London, England). 2022-10-11. PMID:36219306. ptrh2 is necessary for purkinje cell differentiation and survival and its loss recapitulates progressive cerebellar atrophy and ataxia seen in imnepd patients. 2022-10-11 2023-08-14 Not clear
Takehiro Takahashi, Milan Stoiljkovic, Eric Song, Xiao-Bing Gao, Yuki Yasumoto, Eriko Kudo, Fernando Carvalho, Yong Kong, Annsea Park, Marya Shanabrough, Klara Szigeti-Buck, Zhong-Wu Liu, Ashley Kristant, Yalan Zhang, Parker Sulkowski, Peter M Glazer, Leonard K Kaczmarek, Tamas L Horvath, Akiko Iwasak. LINE-1 activation in the cerebellum drives ataxia. Neuron. 2022-09-07. PMID:36070749. we demonstrated that l1 activation in the cerebellum led to purkinje cell dysfunctions and degeneration and was sufficient to cause ataxia. 2022-09-07 2023-08-14 mouse
Brenda Toscano Márquez, Anna A Cook, Max Rice, Alexia Smileski, Kristen Vieira-Lomasney, François Charron, R Anne McKinney, Alanna J Wat. Molecular Identity and Location Influence Purkinje Cell Vulnerability in Autosomal-Recessive Spastic Ataxia of Charlevoix-Saguenay Mice. Frontiers in cellular neuroscience. vol 15. 2021-12-31. PMID:34970120. molecular identity and location influence purkinje cell vulnerability in autosomal-recessive spastic ataxia of charlevoix-saguenay mice. 2021-12-31 2023-08-13 mouse
Carlos Del Pilar, Rafael Lebrón-Galán, Ester Pérez-Martín, Laura Pérez-Revuelta, Carmelo Antonio Ávila-Zarza, José Ramón Alonso, Diego Clemente, Eduardo Weruaga, David Día. The Selective Loss of Purkinje Cells Induces Specific Peripheral Immune Alterations. Frontiers in cellular neuroscience. vol 15. 2021-12-17. PMID:34916910. consequently, a model of human cerebellar degeneration and ataxia -the purkinje cell degeneration (pcd) mouse- has been employed, as it allows the study of different processes of selective neuronal death in the same animal, i.e., purkinje cells in the cerebellum and mitral cells in the olfactory bulb. 2021-12-17 2023-08-13 mouse
Ravi Chopra, Vikram G Shakkotta. Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia. Future neurology. vol 9. issue 2. 2021-10-21. PMID:25221437. translating cerebellar purkinje neuron physiology to progress in dominantly inherited ataxia. 2021-10-21 2023-08-13 mouse
Ravi Chopra, Vikram G Shakkotta. Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia. Future neurology. vol 9. issue 2. 2021-10-21. PMID:25221437. cerebellar ataxia is a symptom of a number of conditions, and the emerging evidence that purkinje neuron dysfunction, in particular, abnormal purkinje neuron repetitive firing, is a major driver of motor dysfunction in a subset of dominantly inherited ataxias is dicussed. 2021-10-21 2023-08-13 mouse
Ravi Chopra, Vikram G Shakkotta. Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia. Future neurology. vol 9. issue 2. 2021-10-21. PMID:25221437. common mechanisms of dysfunction and speculate about potential therapeutic targets, suggesting that purkinje neuron firing abnormalities are a novel target for improving motor dysfunction in patients with some forms of dominantly inherited ataxia are proposed. 2021-10-21 2023-08-13 mouse
Emily A L Wozniak, Zhao Chen, Sharan Paul, Praseuth Yang, Karla P Figueroa, Jill Friedrich, Tyler Tschumperlin, Michael Berken, Melissa Ingram, Christine Henzler, Stefan M Pulst, Harry T Or. Cholecystokinin 1 receptor activation restores normal mTORC1 signaling and is protective to Purkinje cells of SCA mice. Cell reports. vol 37. issue 2. 2021-10-13. PMID:34644575. a sca1 mouse model, pcp2-atxn1[30q]d776, has severe ataxia in absence of progressive purkinje neuron degeneration and death. 2021-10-13 2023-08-13 mouse
Kamyar Zahedi, Marybeth Brooks, Sharon Barone, Negah Rahmati, Tracy Murray Stewart, Matthew Dunworth, Christina Destefano-Shields, Nupur Dasgupta, Steve Davidson, Diana M Lindquist, Christine E Fuller, Roger D Smith, John L Cleveland, Robert A Casero, Manoocher Soleiman. Ablation of polyamine catabolic enzymes provokes Purkinje cell damage, neuroinflammation, and severe ataxia. Journal of neuroinflammation. vol 17. issue 1. 2021-08-19. PMID:33054763. ablation of polyamine catabolic enzymes provokes purkinje cell damage, neuroinflammation, and severe ataxia. 2021-08-19 2023-08-13 Not clear
Lauren N Miterko, Tao Lin, Joy Zhou, Meike E van der Heijden, Jaclyn Beckinghausen, Joshua J White, Roy V Sillito. Neuromodulation of the cerebellum rescues movement in a mouse model of ataxia. Nature communications. vol 12. issue 1. 2021-03-15. PMID:33637754. indeed, genetically eliminating purkinje cell neurotransmission blocked the ability of dbs to reduce ataxia. 2021-03-15 2023-08-13 mouse
Eriola Hoxha, Ilaria Balbo, Maria Concetta Miniaci, Filippo Tempi. Purkinje Cell Signaling Deficits in Animal Models of Ataxia. Frontiers in synaptic neuroscience. vol 10. 2021-02-18. PMID:29760657. purkinje cell signaling deficits in animal models of ataxia. 2021-02-18 2023-08-13 Not clear
Samira Abbasi, Ataollah Abbasi, Yashar Sarbaz, Mahyar Janahmad. Power Spectral Density Analysis of Purkinje Cell Tonic and Burst Firing Patterns From a Rat Model of Ataxia and Riluzole Treated. Basic and clinical neuroscience. vol 8. issue 1. 2020-10-01. PMID:28446951. power spectral density analysis of purkinje cell tonic and burst firing patterns from a rat model of ataxia and riluzole treated. 2020-10-01 2023-08-13 rat