Abstract：
:While selective neuronal death has been an influential theme in Huntington's disease (HD), there is now a preponderance of evidence that significant neuronal dysfunction precedes frank neuronal death. The best evidence for neuronal dysfunction is the observation that gene expression is altered in HD brain, suggesting that transcriptional dysregulation is a central mechanism. Studies of altered gene expression began with careful observations of postmortem human HD brain and subsequently were accelerated by the development of transgenic mouse models. The application of DNA microarray technology has spurred tremendous progress with respect to the altered transcriptional processes that occur in HD, through gene expression studies of both transgenic mouse models as well as cellular models of HD. Gene expression profiles are remarkably comparable across these models, bolstering the idea that transcriptional signatures reflect an essential feature of disease pathogenesis. Finally, gene expression studies have been applied to human HD, thus not only validating the approach of using model systems, but also solidifying the idea that altered transcription is a key mechanism in HD pathogenesis. In the future, gene expression profiling will be used as a readout in clinical trials aimed at correcting transcriptional dysregulation in Huntington's disease.
journal_name
Prog Neurobioljournal_title
Progress in neurobiologyauthors
Cha JHdoi
10.1016/j.pneurobio.2007.03.004subject
Has Abstractpub_date
2007-11-01 00:00:00pages
228-48issue
4eissn
0301-0082issn
1873-5118pii
S0301-0082(07)00071-8journal_volume
83pub_type
杂志文章,评审abstract：:This review suggests that a previously unrecognized spinal cord pathway may be of major importance in the conduction of the somatosensory evoked potential (SEP) from the lower limb in Man. The nerve fiber type activated by a "typical" peripheral nerve stimulus used in studying the SEP will activate the posterior tibia...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/0301-0082(85)90021-8
更新日期：1985-01-01 00:00:00
abstract：:It seems likely that the Alzheimer disease (AD)-related dendritic changes addressed in this article are induced by two principally different processes. One process is linked to the plastic response associated with deafferentation, that is, long-lasting transneuronally induced regressive changes in dendritic geometry a...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(98)00022-7
更新日期：1998-08-01 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：:High frequency oscillations (HFO) have a variety of characteristics: band-limited or broad-band, transient burst-like phenomenon or steady-state. HFOs may be encountered under physiological or under pathological conditions (pHFO). Here we review the underlying mechanisms of oscillations, at the level of cells and netw...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2012.02.005
更新日期：2012-09-01 00:00:00
abstract：:The scientific scenario of amyotrophic lateral sclerosis (ALS) has dramatically changed since TDP-43 aggregates were discovered in 2006 as the main component of the neuronal inclusions seen in the disease, and more recently, when the implication of C9ORF72 expansion in familial and sporadic cases of ALS and frontotemp...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2016.05.004
更新日期：2016-07-01 00:00:00
abstract：:The electrophysiological alterations provoked by axotomy have now been studied for almost half a century, in a number of different cell types. Consequently, it is now possible to detail some common mechanisms underlying these changes and to sort out certain trends in the data. The major phenomena reviewed in this sect...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/0301-0082(90)90039-j
更新日期：1990-01-01 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：: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：:The ubiquitin-proteasome system (UPS) is the major intracellular proteolytic mechanism controlling the degradation of misfolded/abnormal proteins. A common hallmark in amyotrophic lateral sclerosis (ALS) and in other neurodegenerative disorders is the accumulation of misfolded/abnormal proteins into the damaged neuron...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2011.10.001
更新日期：2012-05-01 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：:The scientific understanding of preproglucagon derived peptides has provided people with type 2 diabetes with two novel classes of glucose lowering agents, the dipeptidyl peptidase IV (DPP-IV) inhibitors and GLP-1 receptor agonists. For the scientists, the novel GLP-1 agonists, and DPP-IV inhibitors have evolved as us...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2010.07.003
更新日期：2010-11-01 00:00:00
abstract：:As the population ages, the economic and societal impacts of neurodegenerative and neuropsychiatric disorders are expected to rise sharply. Like dementia, late-life depressive disorders are common and are linked to increased disability, high healthcare utilisation, cognitive decline and premature mortality. Considerab...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2012.05.009
更新日期：2012-07-01 00:00:00
abstract：:Should patients with Parkinson's disease participate in research involving stem cell treatments? Are induced pluripotent stem cells (iPSC) the ethical solution to the moral issues regarding embryonic stem cells? How can we adapt trial designs to best assess small numbers of patients in receipt of invasive experimental...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2013.04.003
更新日期：2013-11-01 00:00:00
abstract：:One of the exciting advances in modern medicine and life science is cell-based neurovascular regeneration of damaged brain tissues and repair of neuronal structures. The progress in stem cell biology and creation of adult induced pluripotent stem (iPS) cells has significantly improved basic and pre-clinical research i...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2017.03.003
更新日期：2017-10-01 00:00:00
abstract：:Preconditioning is a phenomenon in which brief episodes of a sublethal insult induce robust protection against subsequent lethal injuries. Preconditioning has been observed in multiple organisms and can occur in the brain as well as other tissues. Extensive animal studies suggest that the brain can be preconditioned t...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2013.11.005
更新日期：2014-03-01 00:00:00
abstract：:Homeoproteins are well known for their role in defining the shape of organs during early development. The late expression of some homeogenes in the nervous system suggests that they might have other, additional functions, possibly in neurite growth and target recognition. The 60 amino acid-long peptide corresponding t...
journal_title：Progress in neurobiology
pub_type： 杂志文章
doi：10.1016/0301-0082(94)90070-1
更新日期：1994-02-01 00:00:00
abstract：:Immunocytochemical mapping studies employing the extensively used monoclonal anti-muscarinic acetylcholine receptor (mAChR) antibody M35 are reviewed. We focus on three neuronal muscarinic cholinoceptive substrates, which are target regions of the cholinergic basal forebrain system intimately involved in cognitive fun...
journal_title：Progress in neurobiology
pub_type： 历史文章,杂志文章,评审
doi：10.1016/s0301-0082(98)00092-6
更新日期：1999-08-01 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
abstract：:Noradrenaline release in areas within the forebrain occurs following activation of noradrenergic cells in the locus coeruleus (LoC). Release of noradrenaline by attentional/arousal/vigilance factors appears to be essential for learning and is responsible for the consolidation of memory. Noradrenaline can activate any ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(02)00023-0
更新日期：2002-08-01 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：:Retrospective studies in humans suggest that chronic maternal stress during pregnancy, associated with raised plasma levels of CRH, ACTH and cortisol may increase the likelihood of preterm birth, developmental delays and behavioural abnormalities in the children. In adulthood, it may contribute to the significant asso...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(01)00018-1
更新日期：2001-12-01 00:00:00
abstract：:Neuromuscular junctions (NMJs) have long been studied as particularly accessible examples of chemical synapses. Nonetheless, some important features of neuromuscular transmission are still poorly understood. One of these is the low statistical variability of the number of transmitter quanta released from motor nerve t...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2015.09.004
更新日期：2015-11-01 00:00:00
abstract：:The spinocerebellar ataxias type 1 (SCA1), 2 (SCA2), 3 (SCA3), 6 (SCA6) and 7 (SCA7) are genetically defined autosomal dominantly inherited progressive cerebellar ataxias (ADCAs). They belong to the group of CAG-repeat or polyglutamine diseases and share pathologically expanded and meiotically unstable glutamine-encod...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2013.01.001
更新日期：2013-05-01 00:00:00
abstract：:Most neurological diseases have a multifactorial nature and the number of molecular mechanisms discovered as underpinning these diseases is continuously evolving. The old concept of developing selective agents for a single target does not fit with the medical need of most neurological diseases. The development of desi...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2016.03.011
更新日期：2016-07-01 00:00:00
abstract：:The present critical review analyzes the question of how nanoparticles from continuously growing industrial production and use of nanomaterials may impact human brain health. Available evidence suggests incomplete effectiveness of protective barriers of the brain against nanoparticles translocation to the brain. This ...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2017.10.003
更新日期：2018-01-01 00:00:00
abstract：:Chronic restraint stress leads to increases in brain derived neurotrophic factor (BDNF) mRNA and protein in some regions of the brain, e.g. the basal lateral amygdala (BLA) but decreases in other regions such as the CA3 region of the hippocampus and dendritic spine density increases or decreases in line with these cha...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2013.10.005
更新日期：2014-01-01 00:00:00
abstract：:Parkinson's disease was first described in 1817 by James Parkinson. It is one of the most common neurodegenerative disorders, affecting the nigrostriatal pathways involved in the control of movement. Since the introduction of L-DOPA as a therapy in the mid-1960s, clinical management of the symptoms has progressed, but...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2008.05.003
更新日期：2008-08-01 00:00:00
abstract：:The extracellular parasite Trypanosoma brucei causes human African trypanosomiasis (HAT), also known as sleeping sickness. Trypanosomes are transmitted by tsetse flies and HAT occurs in foci in sub-Saharan Africa. The disease, which is invariably lethal if untreated, evolves in a first hemo-lymphatic stage, progressin...
journal_title：Progress in neurobiology
pub_type： 杂志文章
doi：10.1016/j.pneurobio.2009.12.001
更新日期：2010-06-01 00:00:00
abstract：:An important biological consequence of the initial interactions between the cell surface and its extracellular environment is the diversity of cellular responses ranging from overt repulsion or avoidance reaction to stable adhesion or final positioning. It is now evident that positive and negative guiding mechanisms a...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s0301-0082(99)00061-1
更新日期：2000-08-01 00:00:00
abstract：:Over the past two decades, the development of neuroimaging techniques has allowed the non-invasive investigation of neuroplastic changes associated with psychotherapeutic treatment. The aim of the present article is to present a systematic and critical review of longitudinal studies addressing the impact of psychother...
journal_title：Progress in neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.pneurobio.2013.10.006
更新日期：2014-03-01 00:00:00
