ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2001
Increased expression of alpha 1A Ca2+ channel currents arising from expanded trinucleotide repeats in spinocerebellar ataxia type 6.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
What it found
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Who cites it
33 citing papers in PubMed, 100 citations in OpenAlex.
- Resilience to Endoplasmic Reticulum Stress Mitigates Membrane Hyperexcitability Underlying Late Disease Onset in a Murine Model of SCA6.Annals of neurology · 2026Article
- Modulation of Pyruvate Export and Extracellular Pyruvate Concentration in Primary Astrocyte Cultures.Neurochemical research · 2024Article
- Human Induced Pluripotent Stem Cell-Based Modelling of Spinocerebellar Ataxias.Stem cell reviews and reports · 2022Review
- Gain-of-function mutations in the UNC-2/CaV2α channel lead to excitation-dominant synaptic transmission ineLife · 2019Article
- Vestibular Performance During High-Acceleration Stimuli Correlates with Clinical Decline in SCA6.Cerebellum (London, England) · 2015Article
- Molecular mechanism of Spinocerebellar Ataxia type 6: glutamine repeat disorder, channelopathy and transcriptional dysregulation. The multifaceted aspects of a single mutation.Frontiers in cellular neuroscience · 2015Review
- Autosomal dominant cerebellar ataxia type III: a review of the phenotypic and genotypic characteristics.Orphanet journal of rare diseases · 2013Review
- Cytoplasmic location of α1A voltage-gated calcium channel C-terminal fragment (Cav2.1-CTF) aggregate is sufficient to cause cell death.PloS one · 2013Article
- Deranged calcium signaling in Purkinje cells and pathogenesis in spinocerebellar ataxia 2 (SCA2) and other ataxias.Cerebellum (London, England) · 2012Review
- Touch responsiveness in zebrafish requires voltage-gated calcium channel 2.1b.Journal of neurophysiology · 2012Article
- Neuronal P/Q-type calcium channel dysfunction in inherited disorders of the CNS.Nature reviews. Neurology · 2012Review
- Role of inositol 1,4,5-trisphosphate receptors in pathogenesis of Huntington's disease and spinocerebellar ataxias.Neurochemical research · 2011Review
- CaV2.1 channelopathies.Pflugers Archiv : European journal of physiology · 2010Review
- Calcium signaling and neurodegeneration.Acta naturae · 2010Article
- The carboxy-terminal fragment of alpha(1A) calcium channel preferentially aggregates in the cytoplasm of human spinocerebellar ataxia type 6 Purkinje cells.Acta neuropathologica · 2010Article
- Ataxin active site determination using spectral distribution of electron ion interaction potentials of amino acids.Medical & biological engineering & computing · 2010Article
- Different relationship of N- and P/Q-type Ca2+ channels to channel-interacting slots in controlling neurotransmission at cultured hippocampal synapses.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2010Article
- Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2009Article
- Calcium signaling in neurodegeneration.Molecular neurodegeneration · 2009Article
- Calcium signaling and neurodegenerative diseases.Trends in molecular medicine · 2009Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
The expansion of polyglutamine tracts encoded by CAG trinucleotide repeats is a common mutational mechanism in inherited neurodegenerative diseases. Spinocerebellar ataxia type 6 (SCA6), an autosomal dominant, progressive disease, arises from trinucleotide repeat expansions present in the coding region of CACNA1A (chromosome 19p13). This gene encodes alpha(1A), the principal subunit of P/Q-type Ca(2+) channels, which are abundant in the CNS, particularly in cerebellar Purkinje and granule neurons. We assayed ion channel function by introduction of human alpha(1A) cDNAs in human embryonic kidney 293 cells that stably coexpressed beta(1) and alpha(2)delta subunits. Immunocytochemical analysis showed a rise in intracellular and surface expression of alpha(1A) protein when CAG repeat lengths reached or exceeded the pathogenic range for SCA6. This gain at the protein level was not a consequence of changes in RNA stability, as indicated by Northern blot analysis. The electrophysiological behavior of alpha(1A) subunits containing expanded (EXP) numbers of CAG repeats (23, 27, and 72) was compared against that of wild-type subunits (WT) (4 and 11 repeats) using standard whole-cell patch-clamp recording conditions. The EXP alpha(1A) subunits yielded functional ion channels that supported inward Ca(2+) channel currents, with a sharp increase in P/Q Ca(2+) channel current density relative to WT. Our results showed that Ca(2+) channels from SCA6 patients display near-normal biophysical properties but increased current density attributable to elevated protein expression at the cell surface.
Indexed as
Identifiers
11717352PMC6763910W2137724558What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.