Early onset of ataxia in moonwalker mice is accompanied by complete ablation of type II unipolar brush cells and Purkinje cell dysfunction.
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A positive feedback loop linking enhanced mGluR function and basal calcium in spinocerebellar ataxia type 2Do mutations in the murine ataxia gene TRPC3 cause cerebellar ataxia in humans?The mutant Moonwalker TRPC3 channel links calcium signaling to lipid metabolism in the developing cerebellum.Modeling Suggests TRPC3 Hydrogen Bonding and Not Phosphorylation Contributes to the Ataxia Phenotype of the Moonwalker Mouse.α-Synuclein expression in the mouse cerebellum is restricted to VGluT1 excitatory terminals and is enriched in unipolar brush cells.Consensus Paper: Cerebellar Development.The Moonwalker mouse: new insights into TRPC3 function, cerebellar development, and ataxia.TRPC Channels: Prominent Candidates of Underlying Mechanism in Neuropsychiatric Diseases.TRPC3-dependent synaptic transmission in central mammalian neurons.Are Type 1 metabotropic glutamate receptors a viable therapeutic target for the treatment of cerebellar ataxia?Cellular and circuit mechanisms underlying spinocerebellar ataxias.Calcium signaling, PKC gamma, IP3R1 and CAR8 link spinocerebellar ataxias and Purkinje cell dendritic development.Cerebellar modules operate at different frequencies.Commentary on "E. Mugnaini and A. Floris, the unipolar brush cell: a neglected neuron of the mammalian cerebellar cortex. J Comp Neurol, 339:174-180, 1994".Ion channel dysfunction in cerebellar ataxia.Structure-function analyses of the ion channel TRPC3 reveal that its cytoplasmic domain allosterically modulates channel gating
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Early onset of ataxia in moonwalker mice is accompanied by complete ablation of type II unipolar brush cells and Purkinje cell dysfunction.
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article científic
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article scientifique
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bilimsel makale
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scientific article published on December 2013
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Early onset of ataxia in moonw ...... and Purkinje cell dysfunction.
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Early onset of ataxia in moonw ...... and Purkinje cell dysfunction.
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Early onset of ataxia in moonw ...... and Purkinje cell dysfunction.
@en
Early onset of ataxia in moonw ...... and Purkinje cell dysfunction.
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Early onset of ataxia in moonw ...... and Purkinje cell dysfunction.
@en
Early onset of ataxia in moonw ...... and Purkinje cell dysfunction.
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Early onset of ataxia in moonw ...... and Purkinje cell dysfunction
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Enrico Mugnaini
Gabriella Sekerková
Jin-Ah Kim
Lutz Birnbaumer
Maximiliano J Nigro
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19689-19694
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10.1523/JNEUROSCI.2294-13.2013
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2013-12-01T00:00:00Z