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TranscriptomicsVulnerability of Purkinje cells induced from spinocerebellar ataxia type 6 patient-derived iPS cells [1]
Spinocerebellar ataxia type 6 (SCA6) is a dominantly inherited neurodegenerative disease characterized by loss of Purkinje cells in the cerebellum. SCA6 is caused by CAG trinucleotide repeat expansion in CACNA1A, which encodes Cav2.1, ?1A subunit of P/Q-type calcium channel. However, the pathogenic ...
ORGANISM(S): Homo sapiens 
2016-11-08 | E-GEOD-85347 | biostudies-arrayexpress
TranscriptomicsSelective loss of 5hmC links to A-T Purkinje cell vulnerability
This SuperSeries is composed of the SubSeries listed below. The neurodegenerative disease known as ataxia-telangiectasia (A-T) is caused by the absence of the ATM (A-T mutated) protein. A long-standing mystery surrounding A-T is why cerebellar Purkinje cells (PCs) appear uniquely vulnerable to ATM-...
ORGANISM(S): Homo sapiens 
2015-10-30 | E-GEOD-61169 | biostudies-arrayexpress
ProteomicsQuantitative proteomics of immunocaptured mitochondria from Purkinje Cells
Mitochondria vary in morphology and function in different tissues, however little is known about their molecular diversity among cell types. To investigate mitochondrial diversity in vivo, we developed an efficient protocol to isolate cell type-specific mitochondria based on a new MitoTag mouse. We ...
ORGANISM(S): Mus musculus (Mouse) 
2019-09-03 | PXD010772 | Pride
Mitochondria Purkinje cells Neurons 
TranscriptomicsBAC-trap studies of Purkinje cells in normal and FMR1 mutant mice
The molecular mechanism(s) leading to Purkinje neuron loss in the neurodegenerative disorder Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS) are limited by the complex morphology of this cell type. Purkinje neurons are notoriously difficult to isolate and maintain in culture presenting considera...
ORGANISM(S): Mus musculus 
2014-05-01 | E-GEOD-57034 | biostudies-arrayexpress
TranscriptomicsGene expression of cerebellar Purkinje cells
We performed gene-expression analysis of mouse Purkinje cells as a model M-bM-^@M-^\single-type neuronM-bM-^@M-^]. DNA microarray analysis detected at least 7,055 genes in Purkinje cells, most of which are classified into functional molecule categories. Our comparative analysis between Purkinje cell...
ORGANISM(S): Mus musculus 
2013-01-17 | E-GEOD-26749 | biostudies-arrayexpress
GenomicsMeDIP analysis of 5mC and 5hmC marks in human ataxia-telangiectasia cerebellum
Genomic DNA was prepared, fragmented, and immunoprecipitated with antibodies specific for 5mC or 5hmC prior to standard sequencing. The neurodegenerative disease known as ataxia-telangiectasia (A-T) is caused by the absence of the ATM (A-T mutated) protein. A long-standing mystery surrounding A-T i...
ORGANISM(S): Homo sapiens 
2015-10-30 | E-GEOD-61133 | biostudies-arrayexpress
TranscriptomicsAtaxia-telangiectasia cerebellar cortex expression
Cerebellar cortex expression in ataxia-telangiectasia patients and normal controls. The neurodegenerative disease known as ataxia-telangiectasia (A-T) is caused by the absence of the ATM (A-T mutated) protein. A long-standing mystery surrounding A-T is why cerebellar Purkinje cells (PCs) appear uni...
ORGANISM(S): Homo sapiens 
2015-10-30 | E-GEOD-61019 | biostudies-arrayexpress
ModelsMohr2022 - Rabbit Purkinje Cardiac Electrophysiology
we performed a comparative benchmark and investigated a variety of mathematical cardiac AP models, including a newly developed minimalistic model specifically tailored to the AP of rabbit Purkinje cells, for their ability to substitute experiments. The simulated changes in AP duration (dAPD90) at in...
2025-03-24 | BIOMD0000001100 | BioModels
ProteomicsPurkinje cell proteome of the cerebellum of AP-2 cKo
Identification of whole proteome changes in Purkinje cells of the cerebellum of AP-2 cKo and control mice at 2 months of age after APEX AAV injection
ORGANISM(S): Mus musculus (Mouse) 
2024-12-18 | PXD058847 | Pride
Apex Cerebellum Purkinje cells Ap-2 
TranscriptomicsNorth American Brain Expression Consortium: Laser capture microdissection of Cerebellar Purkinje Cells
Previous studies in bulk tissue suggest that there are abundant expression quantitative trait loci (eQTLs) in human brain. This sample series is of cerebellar Purkinje cells isolated using laser capture microdissection from human cases without neurological disease but of known genotypes. These data ...
ORGANISM(S): Homo sapiens 
2013-01-01 | E-GEOD-37205 | biostudies-arrayexpress
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