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Keywords: Cerebellum
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Journal Articles
Journal Articles
Dis Model Mech (2023) 16 (7): dmm050005.
Published: 17 July 2023
... Universidade Federal do Rio Grande do Norte http://dx.doi.org/10.13039/501100008532 Coordenação de Aperfeiçoamento de Pessoal de Nível Superior http://dx.doi.org/10.13039/501100002322 Susceptibility window Calcification Brainstem Cerebellum Blood–brain barrier Neuronal migration...
Journal Articles
Dis Model Mech (2022) 15 (9): dmm049642.
Published: 13 September 2022
... demonstrate that Kctd7 -deficient mice develop seizures and locomotor defects with features similar to those observed in human KCTD7 -associated diseases. We also show that Kctd7 is required for Purkinje cell survival in the cerebellum and that selective degeneration of these neurons is accompanied by defects...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2022) 15 (8): dmm049514.
Published: 5 August 2022
... cerebellar nuclei neurons, the main output of the cerebellum, was altered. The aberrant activity correlated with the onset of ataxia. However, although the severity of ataxia increased with age, the severity of the aberrant cerebellar output was not progressive. The abnormal cerebellar output, however...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (2): dmm040840.
Published: 9 December 2019
.... Based on the relatively well-preserved cytoarchitecture in the mdx cerebellum, our data suggest that faulty signals across the circuit between Purkinje cells and cerebellar nuclei drive the abnormal firing activity. The in vivo requirements of dystrophin during cerebellar circuit communication could...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2018) 11 (9): dmm034181.
Published: 21 September 2018
... conductance calcium-activated potassium (SK) channel activator NS309 into the cerebellum of tottering mice in the midst of an attack normalized the firing of Purkinje cells and aborted attacks. Conversely, we found that inducing high-frequency burst firing of Purkinje cells in wild-type animals is sufficient...
Includes: Supplementary data
Journal Articles
Journal Articles
Dis Model Mech (2017) 10 (11): 1343–1352.
Published: 1 November 2017
...-sulphur cluster formation and ATP production. Frataxin deficiency is associated with mitochondrial dysfunction in FRDA patients and animal models; however, early mitochondrial pathology in FRDA cerebellum remains elusive. Using frataxin knock-in/knockout (KIKO) mice and KIKO mice carrying the mitoDendra...
Includes: Supplementary data