Abstract：
:Huntington's disease (HD) is caused by an expansion of exonic CAG triplet repeats in the gene encoding huntingtin protein (Htt), but the mechanisms by which this mutant protein causes neurodegeneration remain unknown. Here we show that lymphoblast mitochondria from patients with HD have a lower membrane potential and depolarize at lower calcium loads than do mitochondria from controls. We found a similar defect in brain mitochondria from transgenic mice expressing full-length mutant huntingtin, and this defect preceded the onset of pathological or behavioral abnormalities by months. By electron microscopy, we identified N-terminal mutant huntingtin on neuronal mitochondrial membranes, and by incubating normal mitochondria with a fusion protein containing an abnormally long polyglutamine repeat, we reproduced the mitochondrial calcium defect seen in human patients and transgenic animals. Thus, mitochondrial calcium abnormalities occur early in HD pathogenesis and may be a direct effect of mutant huntingtin on the organelle.
journal_name
Nat Neuroscijournal_title
Nature neuroscienceauthors
Panov AV,Gutekunst CA,Leavitt BR,Hayden MR,Burke JR,Strittmatter WJ,Greenamyre JTdoi
10.1038/nn884subject
Has Abstractpub_date
2002-08-01 00:00:00pages
731-6issue
8eissn
1097-6256issn
1546-1726pii
nn884journal_volume
5pub_type
杂志文章abstract：:Sustained activity encoding visual working memory representations has been observed in several cortical areas of primates. Where along the visual pathways this activity emerges remains unknown. Here we show in macaques that sustained spiking activity encoding memorized visual motion directions is absent in direction-s...
journal_title：Nature neuroscience
pub_type： 杂志文章
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更新日期：2014-09-01 00:00:00
abstract：:Transcriptomic analyses of postmortem brains have begun to elucidate molecular abnormalities in autism spectrum disorder (ASD). However, a crucial pathway involved in synaptic development, RNA editing, has not yet been studied on a genome-wide scale. Here we profiled global patterns of adenosine-to-inosine (A-to-I) ed...
journal_title：Nature neuroscience
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abstract：:The projections and odor responses of mammalian olfactory receptor neurons, as well as the physiology of the bulb's principal neurons-the mitral cells (MCs)-are known from studies in slices and anesthetized animals. In behaving rats trained to discriminate between two odors associated with different reinforcers, we ex...
journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/14801
更新日期：1999-11-01 00:00:00
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journal_title：Nature neuroscience
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更新日期：2000-11-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
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更新日期：2006-06-01 00:00:00
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pub_type： 杂志文章
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更新日期：2014-02-01 00:00:00
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更新日期：2007-08-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：2017-12-01 00:00:00
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pub_type： 杂志文章
doi：10.1038/nn1593
更新日期：2006-01-01 00:00:00
abstract：:Stimuli that elicit itch are detected by sensory neurons that innervate the skin. This information is processed by the spinal cord; however, the way in which this occurs is still poorly understood. Here we investigated the neuronal pathways for itch neurotransmission, particularly the contribution of the neuropeptide ...
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doi：10.1038/s41593-018-0119-z
更新日期：2018-05-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/nn.3710
更新日期：2014-06-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/nn.2257
更新日期：2009-02-01 00:00:00
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doi：10.1038/nn.3356
更新日期：2013-05-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/nn.2135
更新日期：2008-07-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/nn.2223
更新日期：2009-01-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/nn.2404
更新日期：2009-11-01 00:00:00
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pub_type： 杂志文章
doi：10.1038/s41593-019-0416-1
更新日期：2019-07-01 00:00:00
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pub_type： 杂志文章
doi：10.1038/nn769
更新日期：2001-12-01 00:00:00
abstract：:Cell type-specific expression of optogenetic molecules allows temporally precise manipulation of targeted neuronal activity. Here we present a toolbox of four knock-in mouse lines engineered for strong, Cre-dependent expression of channelrhodopsins ChR2-tdTomato and ChR2-EYFP, halorhodopsin eNpHR3.0 and archaerhodopsi...
journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/nn.3078
更新日期：2012-03-25 00:00:00
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更新日期：2020-04-01 00:00:00
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更新日期：2017-09-01 00:00:00
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更新日期：2015-05-01 00:00:00
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更新日期：2018-04-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
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更新日期：2013-04-01 00:00:00
abstract：:Most advances in addiction treatment to date have addressed the physical dependence and withdrawal that accompany addiction to some drugs of abuse. In contrast, it has proven more difficult to develop medications that effectively treat drug craving and relapse, the core features of addictive disorders. Current efforts...
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更新日期：2002-11-01 00:00:00
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pub_type： 杂志文章
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更新日期：2014-12-01 00:00:00
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pub_type： 杂志文章,评审
doi：10.1038/nn.4413
更新日期：2016-11-01 00:00:00
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journal_title：Nature neuroscience
pub_type： 杂志文章
doi：10.1038/nn.2945
更新日期：2011-10-23 00:00:00
abstract：:Studies of populations of receptor proteins suggest that their number and location are highly regulated. Single-particle tracking of glycine receptors now reveals the direct movement of receptors between different clusters of the anchoring protein gephyrin. ...
journal_title：Nature neuroscience
pub_type： 评论,杂志文章,评审
doi：10.1038/85042
更新日期：2001-03-01 00:00:00
