Abstract：
:The hereditary ataxias are a group of inherited neurodegenerative disorders characterized by progressive ataxia that results from degeneration of the cerebellum and its afferent and efferent connections. Recent molecular research has led not only to the discovery of a number of causative mutations, but also shed light on the likely mechanisms by which these mutations cause the respective phenotypes. In Friedreich's ataxia (FRDA), the most common type of autosomal recessive ataxia, the loss of a mitochondrial protein, frataxin, results in overload of mitochondrial iron and oxidative stress. The autosomal dominant ataxias, spinocerebellar ataxia type I (SCAI), SCA2, SCA3 and SCA7, are caused by inheritance of an unstable, expanded CAG trinucleotide repeat. These disorders are assumed to be due to a novel deleterious function of the extended polyglutamine sequences within the proteins encoded by the respective genes. Recent observations in transgenic mice and in human post-mortem tissue suggest that the extended proteins are transported into the nucleus of neurons where they form intranuclear inclusions that disrupt normal nuclear function. In another group of dominant disorders, episodic ataxia type I and type 2 (EA-I, EA-2) and SCA6, the mutations affect genes that code for ion channels.
journal_name
Trends Neuroscijournal_title
Trends in neurosciencesauthors
Klockgether T,Evert Bdoi
10.1016/s0166-2236(98)01315-0subject
Has Abstractpub_date
1998-09-01 00:00:00pages
413-8issue
9eissn
0166-2236issn
1878-108Xpii
S0166-2236(98)01315-0journal_volume
21pub_type
杂志文章,评审abstract：:Striatal enriched tyrosine phosphatase (STEP) has recently been identified as a crucial player in the regulation of synaptic function. It is restricted to neurons within the CNS and acts by downregulating the activity of MAP kinases, the tyrosine kinase Fyn and NMDA receptors. By modulating these substrates, STEP acts...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2006.06.007
更新日期：2006-08-01 00:00:00
abstract：:Schizophrenia (SZ) is primarily an adult psychiatric disorder in which disturbances caused by susceptibility genes and environmental insults during early neurodevelopment initiate neurophysiological changes over a long time course, culminating in the onset of full-blown disease nearly two decades later. Aberrant postn...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2009.05.007
更新日期：2009-09-01 00:00:00
abstract：:Speakers of different languages think about time differently in accordance with the spatial metaphors common in their languages. Furthermore, learning new spatial metaphors in language instills new non-linguistic representations of time, suggesting that language can play a causal role in constructing mental timelines....
journal_title：Trends in neurosciences
pub_type： 杂志文章
doi：10.1016/j.tins.2018.08.004
更新日期：2018-10-01 00:00:00
abstract：:Potassium channels are crucial regulators of neuronal excitability, setting resting membrane potentials and firing thresholds, repolarizing action potentials and limiting excitability. Although most of our understanding of K+ channels is based on somatic recordings, there is good evidence that these channels are prese...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2004.02.012
更新日期：2004-04-01 00:00:00
abstract：:Severed distal segments of nerve axons (anucleate axons) have now been reported to survive for weeks to years in representative organisms from most phyla, including the vertebrates. Among invertebrates (especially crustaceans), such long-term survival might involve transfer of proteins from adjacent intact cells to an...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(91)90104-3
更新日期：1991-05-01 00:00:00
abstract：:Mis-sense mutations of presenilin 1 increase the release of amyloidogenic peptide from amyloid precursor protein (APP) and are a major cause of familial Alzheimer's Disease. Loss-of-function mutations of presenilins in the mouse, Caenorhabditis elegans and Drosophila result in severe developmental defects caused by di...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/s0166-2236(99)01455-1
更新日期：1999-10-01 00:00:00
abstract：:Aging is characterized by decrements in maximum function and accumulation of mitochondrial DNA mutations, which are best observed in organs such as the brain that contain post-mitotic cells. Oxygen radicals are increasingly considered responsible for part of these aging changes. Comparative studies of animals with dif...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2004.07.005
更新日期：2004-10-01 00:00:00
abstract：:During prenatal brain development, ion channels are ubiquitous across several cell types, including progenitor cells and migrating neurons but their function has not been clear. In the past, ion channel dysfunction has been primarily studied in the context of postnatal, differentiated neurons that fire action potentia...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2019.12.004
更新日期：2020-02-01 00:00:00
abstract：:Foraging-associated predation risk is a natural problem all prey must face. Fear evolved due to its protective functions, guiding and shaping behaviors that help animals adapt to various ecological challenges. Despite the breadth of risky situations in nature that demand diversity in fear behaviors, contemporary neuro...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2016.04.001
更新日期：2016-06-01 00:00:00
abstract：:Cell replacement therapy has been proposed as a means to replace lost dopaminergic neurons in Parkinson's disease (PD). In most studies, the transplanted cells have been placed within the target site, the striatum, and not within the lesioned site, the substantia nigra, as the adult nigrostriatal pathway was thought t...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2011.01.003
更新日期：2011-03-01 00:00:00
abstract：:In the mammalian central nervous system (CNS), coupling of neurons by gap junctions (i.e., electrical synapses) and the expression of the neuronal gap junction protein, connexin 36 (Cx36), transiently increase during early postnatal development. The levels of both subsequently decline and remain low in the adult, conf...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2012.11.001
更新日期：2013-04-01 00:00:00
abstract：:It has been suggested, on the basis of neuroanatomical and behavioural data, that olfactory discrimination in the rat provides privileged access to higher cognitive processes, involves the acquisition of a learning-set and, importantly, can be used to model human hippocampal function and dysfunction. In this article, ...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(93)90043-l
更新日期：1993-01-01 00:00:00
abstract：:Serotonin is an important neuromodulator associated with a wide range of physiological effects in the central nervous system. The exact mechanisms whereby serotonin influences brain development are not well understood, although studies in invertebrate and vertebrate model organisms are beginning to unravel a regulator...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2010.05.005
更新日期：2010-09-01 00:00:00
abstract：:The production by the pyridine MPTP of a parkinsonian syndrome strikingly similar to the 'idiopathic' disorder, and the paucity of evidence supporting a hereditary or infectious etiology for Parkinson's disease (PD), have stimulated a search for environmental chemicals resembling MPTP that might cause PD. In support o...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(89)90135-5
更新日期：1989-02-01 00:00:00
abstract：:It has become apparent in recent years that the cytoskeleton and its associated proteins play a major role in secretion. This review summarizes recent findings on the cytoskeleton organization and the molecular topology of its regulatory proteins, as well as the dynamic changes that occur in this organelle during secr...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(93)90079-2
更新日期：1993-11-01 00:00:00
abstract：:Rett syndrome is a neurodevelopmental disorder that primarily affects females and is caused by mutations in the methyl-CpG-binding-protein 2 (MECP2) gene. Initially, MeCP2 had been shown to be a repressor of gene transcription. In their 2008 paper, Chahrour and colleagues (DOI: 10.1126/science.1153252) reported that M...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2017.11.005
更新日期：2018-02-01 00:00:00
abstract：:CNS myelin inhibits axonal outgrowth in vitro and is one of several obstacles to functional recovery following spinal cord injury. Central to our current understanding of myelin-mediated inhibition are the membrane protein Nogo and the Nogo-66 receptor (NgR). New findings implicate NgR as a point of convergence in sig...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/S0166-2236(03)00062-6
更新日期：2003-04-01 00:00:00
abstract：:Short-term synaptic plasticity has a key role in information processing in the CNS, whereas memories can be formed through long-lasting changes in synaptic strength. Despite the importance of these phenomena, it remains difficult to determine whether a synaptic modulation is expressed at a presynaptic or postsynaptic ...
journal_title：Trends in neurosciences
pub_type： 杂志文章
doi：10.1016/s0166-2236(99)01520-9
更新日期：2000-03-01 00:00:00
abstract：:Rhythmic motor activity requires coordination of different muscles or muscle groups so that they are all active with the same cycle duration and appropriate phase relationships. The neural mechanisms for such phase coupling in vertebrate locomotion are not known. Swimming in the lamprey is accomplished by the generati...
journal_title：Trends in neurosciences
pub_type： 杂志文章
doi：10.1016/0166-2236(92)90006-t
更新日期：1992-11-01 00:00:00
abstract：:Korsakoff's disease is an amnesic syndrome associated with midline diencephalic and brain stem pathology. A number of neurochemical systems course through or near the loci of brain lesions found postmortem in Korsakoff patients, which has stimulated studies to learn whether these systems are implicated in amnesia. Dat...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(90)90146-2
更新日期：1990-08-01 00:00:00
abstract：:In many neurological and psychiatric disorders, including Alzheimer's disease and schizophrenia, symptoms are present that appear to reflect an essential absence of normal movement, cognition and emotional states. These negative symptoms might reflect fundamental impairments in basic brain mechanisms that underlie goa...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/s0166-2236(00)01626-x
更新日期：2000-09-01 00:00:00
abstract：:Conditioning and extinction of fear have traditionally been viewed as two independent learning processes for encoding representations of contexts or cues (conditioned stimuli, CS), aversive events (unconditioned stimuli, US), and their relationship. Based on the analysis of protein kinase signaling patterns in neurons...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2011.10.003
更新日期：2012-03-01 00:00:00
abstract：:Despite extensive information on many aspects of peptide neurobiology, the links between the behavioral effects of neuropeptides and their actions at the cellular and molecular levels are not fully understood. A pair of insect neuropeptides, the cardioacceleratory peptides (CAPs) of the tobacco hawkmoth Manduca sexta,...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(91)90126-f
更新日期：1991-06-01 00:00:00
abstract：:Neurobiology was a central concern at the Marine Biological Laboratory (MBL), Woods Hole at the end of the last century, when the director Charles Otis Whitman set up a seminar for discussion of research and general issues in 'neurology', which took place annually from 1896 to 1900 and generated intense interest among...
journal_title：Trends in neurosciences
pub_type： 历史文章,杂志文章,评审
doi：10.1016/0166-2236(90)90118-t
更新日期：1990-10-01 00:00:00
abstract：:Advances in understanding the biology of neurotrophic factors and their signaling pathways have provided important insights into the normal growth, differentiation and maintenance of neurons. Stimulated by neuropathological observations and genetic discoveries, studies in cell and animal models of neurodegenerative di...
journal_title：Trends in neurosciences
pub_type： 杂志文章
doi：10.1016/S0166-2236(02)00038-3
更新日期：2003-02-01 00:00:00
abstract：:Neurons are dynamic cells that respond and adapt to stimuli throughout their long postmitotic lives. The structural and functional plasticity of neurons requires the regulated transcription of new gene products, and dysregulation of transcription in either the developing or adult brain impairs cognition. We discuss ho...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2018.02.009
更新日期：2018-05-01 00:00:00
abstract：:Within the past decade intensive molecular genetic research has been carried out in the attempt to pinpoint chromosomal loci that may be linked to neuropsychiatric disorders. Joseph Martin reviews progress made in the application of molecular genetics to the study of diseases such as Huntington's disease, Alzheimer's ...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(89)90051-9
更新日期：1989-04-01 00:00:00
abstract：:Previous studies have demonstrated that oligodendrocytes in the spinal cord and brainstem originate from restricted domains of ventral neuroepithelium under the influence of sonic hedgehog protein (SHH). However, the origin of oligodendrocytes in the telencephalon, the most anterior part of the CNS, has not been estab...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/s0166-2236(02)02145-8
更新日期：2002-05-01 00:00:00
abstract：:Recent results shed new light on how some nervous systems can regenerate after injury while others cannot. Until recently, it was widely believed that the main difference between systems that regenerate and those that do not lies in the normal state of their permissiveness to the regenerating axons. Thus, while nonreg...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/0166-2236(94)90056-6
更新日期：1994-07-01 00:00:00
abstract：:NMDA receptors (NMDARs) are ion channels activated by the excitatory neurotransmitter glutamate and are essential to all aspects of brain function, including learning and memory formation. Missense mutations distributed throughout NMDAR subunits have been associated with an array of neurological disorders. Recent stru...
journal_title：Trends in neurosciences
pub_type： 杂志文章,评审
doi：10.1016/j.tins.2017.01.001
更新日期：2017-03-01 00:00:00
