Abstract：
:Spinocerebellar ataxia type 1 (SCA1) is one an intriguing set of nine neurodegenerative diseases caused by the expansion of a unstable trinucleotide CAG repeat where the repeat is located within the coding of the affected gene, i.e. the polyglutamine (polyQ) diseases. A gain-of-function mechanism for toxicity in SCA1, like the other polyQ diseases, is thought to have a major role in pathogenesis. Yet, the specific nature of this gain-of-function is a matter of considerable discussion. An issue concerns whether toxicity stems from the native or normal function of the affected protein versus a novel function induced by polyQ expansion. For SCA1 considerable evidence is accumulating that pathology is mediated by a polyQ-induced exaggeration of a native function of the host protein Ataxin-1 (ATXN1) and that phosphorylation of S776 regulates its interaction with other cellular protein and thereby function. In addition, this posttranslational modification modulates toxicity of ATXN1 with an expanded polyglutamine.
journal_name
Prog Neurobioljournal_title
Progress in neurobiologyauthors
Orr HTdoi
10.1016/j.pneurobio.2012.04.003subject
Has Abstractpub_date
2012-12-01 00:00:00pages
179-85issue
3eissn
0301-0082issn
1873-5118pii
S0301-0082(12)00051-2journal_volume
99pub_type
杂志文章,评审abstract：:Neurologic disease is a major cause of disability in resource-poor countries and a substantial portion of this disease is due to infections of the CNS. A wide variety of emerging and re-emerging viruses contribute to this disease burden. New emerging infections are commonly due to RNA viruses that have expanded their ...
journal_title：Progress in neurobiology
pub_type： 杂志文章
doi：10.1016/j.pneurobio.2009.12.003
更新日期：2010-06-01 00:00:00
abstract：:Until recently, memory consolidation and storage had been traditionally viewed as a permissive process derived from learning-activated molecular signaling cascades which include activations of the NMDA receptors, CaMKII, PKC, PKA and other kinases, new protein synthesis and CREB-mediated gene expression, and subsequen...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2006.06.004
更新日期：2006-06-01 00:00:00
abstract：:Copper is an important trace element that is required for essential enzymes. However, due to its redox activity, copper can also lead to the generation of toxic reactive oxygen species. Therefore, cellular uptake, storage as well as export of copper have to be tightly regulated in order to guarantee sufficient copper ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2014.01.002
更新日期：2014-05-01 00:00:00
abstract：:Cognitive neuroscientists in the late 20th century began the task of identifying the part(s) of the brain concerned with normal behavior as manifest in the psychological capacities as affective powers, reasoning, behaving purposively and the pursuit of goals, following introduction of the 'functional magnetic resonanc...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2016.07.001
更新日期：2016-01-01 00:00:00
abstract：:We review the neuroanatomical, neurophysiological and functional properties of the mammalian subiculum in this paper. The subiculum is a pivotal structure positioned between the hippocampus proper and entorhinal and other cortices, as well as a range of subcortical structures. It is an under-investigated region that p...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(00)00054-x
更新日期：2001-06-01 00:00:00
abstract：:The proteolytical processing of the amyloid precursor protein (APP) gives rise to beta-amyloid peptides, which accumulate in brains of Alzheimer's disease (AD) patients. Different soluble or insoluble higher molecular weight forms of beta-amyloid peptides have been postulated to trigger a complex pathological cascade ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2006.06.001
更新日期：2006-06-01 00:00:00
abstract：:White matter (WM) plasticity during adulthood is a recently described phenomenon by which experience can shape brain structure. It has been observed in humans using diffusion tensor imaging (DTI) and myelination has been suggested as a possible mechanism. Here, we set out to identify molecular and cellular changes ass...
journal_title：Progress in neurobiology
pub_type： 杂志文章
doi：10.1016/j.pneurobio.2020.101770
更新日期：2020-04-01 00:00:00
abstract：:The K+-Cl- co-transporter KCC2 is a neuron-specific, Cl- extruder that uses K+ gradient for maintaining low intracellular [Cl-]. It is indeed well established that sustaining an outwardly-directed electrochemical Cl- gradient across the neuronal membrane is fundamental for a proper function of postsynaptic GABAA recep...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2017.11.002
更新日期：2018-03-01 00:00:00
abstract：:Gene expression patterns in neurons are complex and are modulated in response to multiple extracellular stimuli. In addition, during development and as neurons differentiate into distinct neuronal phenotypes, there is a co-ordinated activation and repression of a variety of genes. It is becoming increasingly evident t...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(96)00041-x
更新日期：1996-11-01 00:00:00
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journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2017.03.010
更新日期：2017-06-01 00:00:00
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journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(98)00029-x
更新日期：1998-10-01 00:00:00
abstract：:Psychosocial and biological factors have been implicated in paedophilia, such as alterations in brain structure and function. The purpose of this paper is to review the expanding body of literature on this topic including brain abnormality case reports, as well as structural and functional neuroimaging studies. Case s...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2014.07.005
更新日期：2014-11-01 00:00:00
abstract：:Coherent high-frequency neuronal activity has been proposed as a physiological indicator of perceptual and higher cognitive processes. Some of these processes can only be investigated in humans and the use of non-invasive recording techniques appears to be a prerequisite for investigating their physiological substrate...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(97)00023-3
更新日期：1997-08-01 00:00:00
abstract：:The advent of proteomics techniques has been enthusiastically accepted in most areas of biology and medicine. In neuroscience, a host of applications was proposed ranging from neurotoxicology, neurometabolism, determination of the proteome of the individual brain areas in health and disease, to name a few. Only recent...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(03)00036-4
更新日期：2003-02-01 00:00:00
abstract：:Narcolepsy is a neurological disorder that afflicts 1 in 2000 individuals and is characterized by excessive daytime sleepiness and cataplexy-a sudden loss of muscle tone triggered by positive emotions. Features of narcolepsy include dysregulation of arousal state boundaries as well as autonomic and metabolic disturban...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2015.12.002
更新日期：2017-05-01 00:00:00
abstract：:At the psychological level, the notion that emotional events may be better remembered is a long accepted view. Its translation into neurobiological mechanisms has led to the proposal of the 'emotional tag' concept, according to which, the activation of the amygdala by emotionality would result in modulation of neural ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2011.03.004
更新日期：2011-06-01 00:00:00
abstract：:Once it was believed that the subthalamic nucleus (STN) was no more than a relay station serving as a "gate" for ascending basal ganglia-thalamocortical circuits. Nowadays, the STN is considered to be one of the main regulators of motor function related to the basal ganglia. The role of the STN in the regulation of as...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2005.09.005
更新日期：2005-08-01 00:00:00
abstract：:After a long period of inconclusive observations, the intrinsic primary afferent neurons of the intestine have been identified. The intestine is thus equipped with two groups of afferent neurons, those with cell bodies in cranial and dorsal root ganglia, and these recently identified afferent neurons with cell bodies ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(97)00051-8
更新日期：1998-01-01 00:00:00
abstract：:This article reviews a series of experiments aimed at investigating the reciprocal trophic interactions which regulate the normal morphofunctional features and the plasticity of the adult rodent climbing fibre-Purkinje cell system. Climbing fibre deprivation induces profound functional and structural changes in the Pu...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：
更新日期：1995-11-01 00:00:00
abstract：:Neural stem and progenitor cells (NSPCs) are the primary source of new neurons in the brain and serve critical roles in tissue homeostasis and plasticity throughout life. Within the vertebrate brain, NSPCs are located within distinct neurogenic niches differing in their location, cellular composition, and proliferativ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2018.06.004
更新日期：2018-11-01 00:00:00
abstract：:Firstly, the noetic value of the ontogenetic approach to the problems of learning and memory is emphasized; then the heterochrony and uneven time course of the development of neural systems are accentuated, which fully holds for the basic cognitive functions. Contrary to a broadly accepted opinion, that inhibitory lea...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(97)00036-1
更新日期：1997-11-01 00:00:00
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journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2010.01.008
更新日期：2010-06-01 00:00:00
abstract：:Alzheimer's Disease (AD) is a highly complex disease involving a broad range of clinical, cellular, and biochemical manifestations that are currently not understood in combination. This has led to many views of AD, e.g. the amyloid, tau, presenilin, oxidative stress, and metal hypotheses. The amyloid hypothesis has do...
journal_title：Progress in neurobiology
pub_type： 杂志文章,meta分析,评审
doi：10.1016/j.pneurobio.2016.06.004
更新日期：2016-08-01 00:00:00
abstract：:The brain circuitry of saccadic eye movements, from brainstem to cortex, has been extensively studied during the last 30 years. The wealth of data gathered allowed the conception of numerous computational models. These models proposed descriptions of the putative mechanisms generating this data, and, in turn, made pre...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2005.11.001
更新日期：2005-11-01 00:00:00
abstract：:Recent evidence has shown that neuroinflammation plays a key role in the pathogenesis of Parkinson's disease (PD). However, different components of the brain's immune system may exert diverse effects on neuroinflammatory events in PD. The adaptive immune response, especially the T cell response, can trigger type 1 pro...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2018.08.002
更新日期：2018-10-01 00:00:00
abstract：:Memorizing new facts and events means that entering information produces specific physical changes within the brain. According to the commonly accepted view, traces of memory are stored through the structural modifications of synaptic connections, which result in changes of synaptic efficiency and, therefore, in forma...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2006.09.004
更新日期：2006-10-01 00:00:00
abstract：:The wide tissue distribution of butyrylcholinesterase (BChE) in organisms makes specific roles possible, although no clear physiologic function has yet been assigned to this enzyme. In vertebrates, it appears e.g. in serum, hemopoietic cells, liver, lung, heart, at cholinergic synapses, in the central nervous system. ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(99)00047-7
更新日期：2000-04-01 00:00:00
abstract：:Several lines of evidence have suggested that mitochondrial dysfunction plays a key role in neurodegeneration. The mitochondrial function is a potential target for steroid hormones, which could exert protective activities in the brain and other tissues. The decrease of some sex steroids with aging has been associated ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2019.03.001
更新日期：2019-05-01 00:00:00
abstract：:Brain lactate formation, intercellular exchange and utilization has been implicated in memory formation. However, the individual role of either neuronal or astroglial monocarboxylate transporters for the acquisition and consolidation of information remains incomplete. Using novel transgenic mice and a viral vector app...
journal_title：Progress in neurobiology
pub_type： 杂志文章
doi：10.1016/j.pneurobio.2020.101888
更新日期：2020-11-01 00:00:00
abstract：:Recognition is the process by which a subject is aware that a stimulus has been previously experienced. It requires that the characteristics of events are perceived, discriminated, identified and then compared (matched) against a memory of the characteristics of previously experienced events. Understanding recognition...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(97)00060-9
更新日期：1998-02-01 00:00:00
