Abstract:
:Huntington's Disease (HD) is a genetic neurodegenerative disease caused by a CAG expansion in the gene encoding Huntingtin (Htt). It is characterized by chorea, cognitive, and psychiatric disorders. The most affected brain region is the striatum, and the clinical symptoms are directly correlated to the rate of striatal degeneration. The wild-type Htt is a ubiquitous protein and its deletion is lethal. Mutated (expanded) Htt produces excitotoxicity, mitochondrial dysfunctions, axonal transport deficit, altered proteasome activity, and gene dysregulation. Transcriptional dysregulation occurs at early neuropathological stages in HD patients. Multiple genes are dysregulated, with overlaps of altered transcripts between mouse models of HD and patient brains. Nuclear localization of Exp-Htt interferes with transcription factors, co-activators, and proteins of the transcriptional machinery. Another key mechanism described so far, is an alteration of cytoplasmic retention of the transcriptional repressor REST, which is normally associated with wild-type Htt. As such, Exp-Htt causes alteration of transcription of multiple genes involved in neuronal survival, plasticity, signaling, and mitochondrial biogenesis and respiration. Besides these transcriptional dysregulations, Exp-Htt affects the chromatin structure through altered post-translational modifications (PTM) of histones and methylation of DNA. Multiple alterations of histone PTM are described, including acetylation, methylation, ubiquitylation, polyamination, and phosphorylation. Exp-Htt also affects the expression and regulation of non-coding microRNAs (miRNAs). First multiple neural miRNAs are controlled by REST, and dysregulated in HD, with concomitant de-repression of downstream mRNA targets. Second, Exp-Htt protein or RNA may also play a major role in the processing of miRNAs and hence pathogenesis. These pleiotropic effects of Exp-Htt on gene expression may represent seminal deleterious effects in the pathogenesis of HD.
journal_name
Front Neuroljournal_title
Frontiers in neurologyauthors
Moumné L,Betuing S,Caboche Jdoi
10.3389/fneur.2013.00127subject
Has Abstractpub_date
2013-10-23 00:00:00pages
127issn
1664-2295journal_volume
4pub_type
杂志文章,评审abstract::A hand exoskeleton driven by myoelectric pattern recognition was designed for stroke rehabilitation. It detects and recognizes the user's motion intent based on electromyography (EMG) signals, and then helps the user to accomplish hand motions in real time. The hand exoskeleton can perform six kinds of motions, includ...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2017.00107
更新日期:2017-03-20 00:00:00
abstract::Background: Agoraphobia was described in 1871 as a condition of fear-related alterations in spatial orientation and locomotor control triggered by places or situations that might cause a patient to panic and feel trapped. In contemporary nosology, however, this original concept of agoraphobia was split into two diagno...
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pub_type: 杂志文章
doi:10.3389/fneur.2019.00874
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abstract::Background: Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease. The clinical manifestations of NIID are complex and easily misdiagnosed. Based on the current knowledge of this disease, it is usually chronic, with almost no acute cases. Stroke-like disease is an extremely rare type of NI...
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journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00529
更新日期:2017-10-17 00:00:00
abstract::Mitochondrial complex III deficiency nuclear type 2 is an autosomal-recessive disorder caused by mutations in TTC19 gene. TTC19 is involved in the preservation of mitochondrial complex III, which is responsible for transfer of electrons from reduced coenzyme Q to cytochrome C and thus, contributes to the formation of ...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2019.00944
更新日期:2019-09-04 00:00:00
abstract::Objective: We conducted this study to analyze the clinical characteristics of the psychiatric symptoms of patients with anti-NMDAR encephalitis. Methods: A retrospective study of anti-NMDAR encephalitis in China was performed. The clinical characteristics of the psychiatric symptoms, the relationship between the antib...
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pub_type: 杂志文章
doi:10.3389/fneur.2019.01330
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abstract::Introduction: Eslicarbazepine acetate (ESL) is an antiepileptic drug approved as monotherapy or add-on for the treatment of epilepsy with seizures of focal onset. ESL owns a good profile in terms of efficacy and tolerability, but its effects on EEG activity and connectivity are unknown. The purpose of this study was t...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2018.01054
更新日期:2018-12-11 00:00:00
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journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2018.00980
更新日期:2018-11-22 00:00:00
abstract::Traumatic brain injury is the leading cause of death in children. Children with severe TBI are in need of neurointensive care where the goal is to prevent secondary brain injury by avoiding secondary insults. Monitoring of cerebral blood flow (CBF) and autoregulation in the injured brain is crucial. However, there are...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2020.00274
更新日期:2020-04-16 00:00:00
abstract::Different subtypes of Alzheimer's disease (AD) with characteristic distributions of neurofibrillary tangles and corresponding brain atrophy patterns have been identified using structural magnetic resonance imaging (MRI). However, the underlying biological mechanisms that determine this differential expression of neuro...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00524
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abstract::We previously reported that persistent application of the non-specific cholinergic agonist carbachol (CAR) increased the frequency of calcium channel-mediated oscillatory activity in pedunculopontine nucleus (PPN) neurons, which we identified as dependent on voltage-gated, high-threshold P/Q-type channels. Here, we te...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2013.00176
更新日期:2013-11-06 00:00:00
abstract:BACKGROUND:Parasympathetic innervation of meninges and ability of carbachol, acetylcholine (ACh) receptor (AChR) agonist, to induce headaches suggests contribution of cholinergic mechanisms to primary headaches. However, neurochemical mechanisms of cholinergic regulation of peripheral nociception in meninges, origin pl...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00163
更新日期:2017-04-27 00:00:00
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journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2013.00170
更新日期:2013-11-01 00:00:00
abstract::Growing evidence shows the bidirectional interactions between sleep, circadian rhythm, and epilepsy. Comprehending how these interact with each other may help to advance our understanding of the pathophysiology of epilepsy and develop new treatment strategies to improve seizure control by reducing the medication side ...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2020.00327
更新日期:2020-05-08 00:00:00
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journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2013.00185
更新日期:2013-11-14 00:00:00
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journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00620
更新日期:2017-11-21 00:00:00
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journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2012.00105
更新日期:2012-06-28 00:00:00
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journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2019.00323
更新日期:2019-04-24 00:00:00
abstract::Objective: To predict psychological distress at 2 months for patients with mild traumatic brain injury. Method: A prospective cohort study of 162 patients with mild traumatic brain injury (MTBI) admitted consecutively to an outpatient clinic at Haukeland University Hospital, Norway. Demographic data were obtained from...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00639
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journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.00102
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journal_title:Frontiers in neurology
pub_type: 杂志文章
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pub_type: 杂志文章
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journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00358
更新日期:2017-07-24 00:00:00
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journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
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journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
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doi:10.3389/fneur.2020.00269
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pub_type: 杂志文章
doi:10.3389/fneur.2020.00201
更新日期:2020-04-03 00:00:00
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pub_type: 杂志文章
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pub_type: 杂志文章,评审
doi:10.3389/fneur.2018.00577
更新日期:2018-07-16 00:00:00