All Relations between cerebellum purkinje cell and Ataxia

Publication Sentence Publish Date Extraction Date Species
Guillermo Philipps, Elizabeth D Tate, Michael R Pranzatell. Intensive Combination Immunotherapy and Neuroinflammation Resolution in a Child With Anti-PCA-1 (Yo) Paraneoplastic Syndrome and 2 Malignancies. Child neurology open. vol 5. 2020-10-01. PMID:30288393. when her severe ataxia progressed, neuroinflammation was characterized by high cerebrospinal fluid purkinje cell cytoplasmic antibody type 1 titers, oligoclonal bands, and neurofilament light chain. 2020-10-01 2023-08-13 Not clear
Man Ding, Chao Weng, Shanghua Fan, Qian Cao, Zuneng L. Purkinje Cell Degeneration and Motor Coordination Deficits in a New Mouse Model of Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay. Frontiers in molecular neuroscience. vol 10. 2020-09-29. PMID:28588446. purkinje cell degeneration and motor coordination deficits in a new mouse model of autosomal recessive spastic ataxia of charlevoix-saguenay. 2020-09-29 2023-08-13 mouse
Vanessa Hull, Yan Wang, Travis Burns, Sheng Zhang, Sarah Sternbach, Jennifer McDonough, Fuzheng Guo, David Pleasur. Antisense Oligonucleotide Reverses Leukodystrophy in Canavan Disease Mice. Annals of neurology. vol 87. issue 3. 2020-08-03. PMID:31925837. we now demonstrate that inhibiting naa synthesis by intracisternal administration of a locked nucleic acid antisense oligonucleotide to young-adult aspartoacylase-deficient mice reverses their pre-existing ataxia and diminishes cerebellar and thalamic vacuolation and purkinje cell dendritic atrophy. 2020-08-03 2023-08-13 mouse
Marliawaty I Gusti Bagus, Clayton Gordy, Rosario Sanchez-Gonzalez, Michael Strupp, Hans Strak. Impact of 4-aminopyridine on vestibulo-ocular reflex performance. Journal of neurology. vol 266. issue Suppl 1. 2020-02-10. PMID:31270663. clinically and pharmacologically, the observed 4-ap-mediated increase of peripheral vestibular input under resting and dynamic conditions can contribute to the observed therapeutic effects of 4-ap in downbeat and upbeat nystagmus as well as episodic ataxia type 2, by an indirect increase of cerebellar purkinje cell discharge. 2020-02-10 2023-08-13 xenopus_laevis
Visou Ady, Brenda Toscano-Márquez, Moushumi Nath, Philip K Chang, Jeanette Hui, Anna Cook, François Charron, Roxanne Larivière, Bernard Brais, R Anne McKinney, Alanna J Wat. Altered synaptic and firing properties of cerebellar Purkinje cells in a mouse model of ARSACS. The Journal of physiology. vol 596. issue 17. 2019-12-11. PMID:29928778. autosomal recessive spastic ataxia of charlevoix-saguenay (arsacs) is an early-onset neurodegenerative human disease characterized in part by ataxia and purkinje cell loss in anterior cerebellar lobules. 2019-12-11 2023-08-13 mouse
Visou Ady, Brenda Toscano-Márquez, Moushumi Nath, Philip K Chang, Jeanette Hui, Anna Cook, François Charron, Roxanne Larivière, Bernard Brais, R Anne McKinney, Alanna J Wat. Altered synaptic and firing properties of cerebellar Purkinje cells in a mouse model of ARSACS. The Journal of physiology. vol 596. issue 17. 2019-12-11. PMID:29928778. our results show that both synaptic and intrinsic alterations in purkinje cell properties likely contribute to disease manifestation in arsacs; these findings resemble pathophysiological changes reported in several other ataxias. 2019-12-11 2023-08-13 mouse
Maxime W C Rousseaux, Tyler Tschumperlin, Hsiang-Chih Lu, Elizabeth P Lackey, Vitaliy V Bondar, Ying-Wooi Wan, Qiumin Tan, Carolyn J Adamski, Jillian Friedrich, Kirk Twaroski, Weili Chen, Jakub Tolar, Christine Henzler, Ajay Sharma, Aleksandar Bajić, Tao Lin, Lisa Duvick, Zhandong Liu, Roy V Sillitoe, Huda Y Zoghbi, Harry T Or. ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism. Neuron. vol 97. issue 6. 2019-07-15. PMID:29526553. importantly, loss of cic in the cerebellum does not cause ataxia or purkinje cell degeneration. 2019-07-15 2023-08-13 Not clear
Hannah Hayhurst, Maria-Eleni Anagnostou, Helen J Bogle, John P Grady, Robert W Taylor, Laurence A Bindoff, Robert McFarland, Doug M Turnbull, Nichola Z La. Dissecting the neuronal vulnerability underpinning Alpers' syndrome: a clinical and neuropathological study. Brain pathology (Zurich, Switzerland). vol 29. issue 1. 2019-05-20. PMID:30021052. we believe interneuron and purkinje cell pathology underpins the clinical development of seizures and ataxia seen in alpers' syndrome. 2019-05-20 2023-08-13 Not clear
Lorenzo Mari, Kaspar Matiasek, Christopher A Jenkins, Alberta De Stefani, Sally L Ricketts, Oliver Forman, Luisa De Risi. Hereditary ataxia in four related Norwegian Buhunds. Journal of the American Veterinary Medical Association. vol 253. issue 6. 2019-05-13. PMID:30179085. clinical relevance findings for the dogs of this report suggested the existence of a hereditary form of ataxia in norwegian buhunds with histologic characteristics suggestive of purkinje cell dysfunction. 2019-05-13 2023-08-13 Not clear
Alexander S Brown, Pratap Meera, Banu Altindag, Ravi Chopra, Emma M Perkins, Sharan Paul, Daniel R Scoles, Eric Tarapore, Jessica Magri, Haoran Huang, Mandy Jackson, Vikram G Shakkottai, Thomas S Otis, Stefan M Pulst, Scott X Atwood, Anthony E Or. MTSS1/Src family kinase dysregulation underlies multiple inherited ataxias. Proceedings of the National Academy of Sciences of the United States of America. vol 115. issue 52. 2019-03-22. PMID:30530649. treatment of each sca model with a clinically approved src inhibitor corrects purkinje neuron basal firing and delays ataxia progression in mtss1 mutants. 2019-03-22 2023-08-13 mouse
Alexander S Brown, Pratap Meera, Banu Altindag, Ravi Chopra, Emma M Perkins, Sharan Paul, Daniel R Scoles, Eric Tarapore, Jessica Magri, Haoran Huang, Mandy Jackson, Vikram G Shakkottai, Thomas S Otis, Stefan M Pulst, Scott X Atwood, Anthony E Or. MTSS1/Src family kinase dysregulation underlies multiple inherited ataxias. Proceedings of the National Academy of Sciences of the United States of America. vol 115. issue 52. 2019-03-22. PMID:30530649. our results identify a common sca therapeutic target and demonstrate a key role for mtss1/sfk in purkinje neuron survival and ataxia progression. 2019-03-22 2023-08-13 mouse
Gustavo A Higuera, Grazia Iaffaldano, Meiwand Bedar, Guy Shpak, Robin Broersen, Shashini T Munshi, Catherine Dupont, Joost Gribnau, Femke M S de Vrij, Steven A Kushner, Chris I De Zeeu. An expandable embryonic stem cell-derived Purkinje neuron progenitor population that exhibits in vivo maturation in the adult mouse cerebellum. Scientific reports. vol 7. issue 1. 2019-03-21. PMID:28821816. many forms of ataxia result from degeneration of cerebellar purkinje cells, but thus far it has not been possible to efficiently generate purkinje neuron (pn) progenitors from human or mouse pluripotent stem cells, let alone to develop a methodology for in vivo transplantation in the adult cerebellum. 2019-03-21 2023-08-13 mouse
Esra Tara, Ariel Vitenzon, Ellen Hess, Kamran Khodakha. Aberrant cerebellar Purkinje cell activity as the cause of motor attacks in a mouse model of episodic ataxia type 2. Disease models & mechanisms. vol 11. issue 9. 2019-03-18. PMID:30279196. aberrant cerebellar purkinje cell activity as the cause of motor attacks in a mouse model of episodic ataxia type 2. 2019-03-18 2023-08-13 mouse
Esra Tara, Ariel Vitenzon, Ellen Hess, Kamran Khodakha. Aberrant cerebellar Purkinje cell activity as the cause of motor attacks in a mouse model of episodic ataxia type 2. Disease models & mechanisms. vol 11. issue 9. 2019-03-18. PMID:30279196. many cerebellar-induced neurological disorders, such as ataxias and cerebellar-induced dystonias, are associated with abnormal purkinje cell activity. 2019-03-18 2023-08-13 mouse
Stephanie De Munter, Dorien Bamps, Ana Rita Malheiro, Ritesh Kumar Baboota, Pedro Brites, Myriam Bae. Autonomous Purkinje cell axonal dystrophy causes ataxia in peroxisomal multifunctional protein-2 deficiency. Brain pathology (Zurich, Switzerland). vol 28. issue 5. 2019-03-05. PMID:29341299. autonomous purkinje cell axonal dystrophy causes ataxia in peroxisomal multifunctional protein-2 deficiency. 2019-03-05 2023-08-13 mouse
Stephanie De Munter, Dorien Bamps, Ana Rita Malheiro, Ritesh Kumar Baboota, Pedro Brites, Myriam Bae. Autonomous Purkinje cell axonal dystrophy causes ataxia in peroxisomal multifunctional protein-2 deficiency. Brain pathology (Zurich, Switzerland). vol 28. issue 5. 2019-03-05. PMID:29341299. we show that selective loss of mfp2 from mature cerebellar purkinje neurons causes a late-onset motor phenotype and progressive purkinje cell degeneration, thereby mimicking ataxia and cerebellar deterioration in patients with mild hsd17b4 mutations. 2019-03-05 2023-08-13 mouse
Ravi Chopra, Aaron H Wasserman, Stefan M Pulst, Chris I De Zeeuw, Vikram G Shakkotta. Protein kinase C activity is a protective modifier of Purkinje neuron degeneration in cerebellar ataxia. Human molecular genetics. vol 27. issue 8. 2019-02-08. PMID:29432535. prkcg has also been identified as an important node in ataxia gene networks more broadly, but the functional role of pkc in other forms of ataxia remains unexplored, and the mechanisms by which pkc isozymes regulate purkinje neuron health are not well understood. 2019-02-08 2023-08-13 mouse
Ravi Chopra, David D Bushart, Vikram G Shakkotta. Dendritic potassium channel dysfunction may contribute to dendrite degeneration in spinocerebellar ataxia type 1. PloS one. vol 13. issue 5. 2018-11-26. PMID:29847609. purkinje neuron dendritic degeneration precedes cell loss in cerebellar ataxia, but the basis for dendritic vulnerability in ataxia remains poorly understood. 2018-11-26 2023-08-13 mouse
Chunyi Liu, Mei Mei, Qiuling Li, Peristera Roboti, Qianqian Pang, Zhengzhou Ying, Fei Gao, Martin Lowe, Shilai Ba. Loss of the golgin GM130 causes Golgi disruption, Purkinje neuron loss, and ataxia in mice. Proceedings of the National Academy of Sciences of the United States of America. vol 114. issue 2. 2018-04-09. PMID:28028212. loss of the golgin gm130 causes golgi disruption, purkinje neuron loss, and ataxia in mice. 2018-04-09 2023-08-13 mouse
Chunyi Liu, Mei Mei, Qiuling Li, Peristera Roboti, Qianqian Pang, Zhengzhou Ying, Fei Gao, Martin Lowe, Shilai Ba. Loss of the golgin GM130 causes Golgi disruption, Purkinje neuron loss, and ataxia in mice. Proceedings of the National Academy of Sciences of the United States of America. vol 114. issue 2. 2018-04-09. PMID:28028212. these cellular defects manifest as reduced cerebellar size and purkinje cell number, leading to ataxia. 2018-04-09 2023-08-13 mouse