Abstract:
:The genetically heterozygous spinocerebellar ataxias are all characterized by cerebellar atrophy and pervasive Purkinje Cell degeneration. Up to date, more than 35 functionally diverse spinocerebellar ataxia genes have been identified. The main question that remains yet unsolved is why do some many genetic insults lead to Purkinje Cell degeneration and spinocerebellar ataxia? To address this question it is important to identify intrinsic pathways important for Purkinje Cell function and survival. In this review, we discuss the current consensus on shared mechanisms underlying the pervasive Purkinje Cell loss in spinocerebellar ataxia. Additionally, using recently published cell type specific expression data, we identified several Purkinje Cell-specific genes and discuss how the corresponding pathways might underlie the vulnerability of Purkinje Cells in response to the diverse genetic insults causing spinocerebellar ataxia.
journal_name
Neurosci Lettjournal_title
Neuroscience lettersauthors
Huang M,Verbeek DSdoi
10.1016/j.neulet.2018.02.004subject
Has Abstractpub_date
2019-01-01 00:00:00pages
49-57eissn
0304-3940issn
1872-7972pii
S0304-3940(18)30080-6journal_volume
688pub_type
杂志文章,评审abstract::The effect of preconditioning (PC) on the changes of nerve growth factor-induced gene A (NGFI-A) expression induced by severe hypobaric hypoxia was studied by in situ hybridization. A PC consisted of three trials of mild hypobaric hypoxia (360 Torr, 2 h) spaced at 24 h. The last trial was followed by severe hypoxia (S...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(02)00577-3
更新日期:2002-08-23 00:00:00
abstract::The effects of the group III mGluR agonist, L-2-amino-4-phosphonobutyrate (L-AP4), on depolarization-stimulated cGMP levels in adult rat cerebellar slices were determined. L-AP4 elicited a concentration-dependent, complete inhibition of cGMP formation stimulated by 4-aminopyridine (4-AP; 1 mM), yielding an IC50 value ...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(97)00402-3
更新日期:1997-06-13 00:00:00
abstract::Intracellular recordings were made from astrocytes of the hippocampal CA1 region in rat slice preparations. A single stimulus at the Schaffer collaterals with high intensities (>/=10 V), but not low intensities (<10 V), induced a slow depolarization similar to the evoked postsynaptic potentials observed in hippocampal...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(03)00308-2
更新日期:2003-06-05 00:00:00
abstract::Here we examine hypothesis that short-term peripheral ZnSO(4)-induced anosmia can produce effects on c-fos expression within spinal cord and caudal medulla in male Wistar rats (n=4). Fos-like-immunoreactive cells revealed by avidin-biotin-peroxidase method show a significant bilateral increase in the nucleus proprius ...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(01)01623-8
更新日期:2001-03-30 00:00:00
abstract::Brain derived neurotrophic factor (BDNF), known to promote survival of neurons, has recently been involved in the regulation of endothelial cells and was shown to possess immunoregulatory properties. We therefore asked whether BDNF has immunoregulating effects in human cerebral endothelial cells (HCEC). Adult HCEC exp...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(02)00778-4
更新日期:2002-09-20 00:00:00
abstract::We reported here the functional characteristics of Na+ -dependent neutral amino acid transport system A in normal human astrocytes and its adaptive regulation, a process in which amino acid starvation induces the transport activity. Reverse transcription-PCR revealed that the system A transporter subtype, SNAT2/ATA2, ...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2004.12.030
更新日期:2005-04-18 00:00:00
abstract::Schnurri-2 (Shn-2) knockout (KO) mice have been proposed as a preclinical neuroinflammatory schizophrenia model. We used behavioral studies and imaging markers that can be readily translated to human populations to explore brain effects of inflammation. Shn-2 KO mice and their littermate control mice were imaged with ...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2015.10.037
更新日期:2015-11-16 00:00:00
abstract::Parkin is a ubiquitin ligase involved in the ubiquitin-proteasome system. Elevating parkin expression in cells reduces markers of oxidative stress while blocking parkin expression increases oxidative stress. In parkin gene knock down mouse and fly models, mitochondria function is deficient. Parkin is neuroprotective a...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2006.03.001
更新日期:2006-06-19 00:00:00
abstract::This study investigated differences in the neural time-course of cognitive conflict and emotional conflict control, using event-related potentials (ERPs). Although imaging studies have provided some evidence that distinct, dissociable neural systems underlie emotional and nonemotional conflict resolution, no ERP study...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2016.03.020
更新日期:2016-04-21 00:00:00
abstract::Analyses of neural mechanisms of duration processing are essential for the understanding of psychological phenomena which evolve in time. Different mechanisms are presumably responsible for the processing of shorter (below 500 ms) and longer (above 500 ms) events but have not yet been a subject of an investigation wit...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2009.12.040
更新日期:2010-01-29 00:00:00
abstract::Interleukin-1 (IL-1) mediates numerous responses on the mouse anterior pituitary cell line AtT-20. We have studied the ligand binding properties of the IL-1 receptors (IL-1R) of the AtT-20 cells and found that they possess 1.5 x 10(3) receptors/cell with a Kd of 0.15 nM for [125I]IL-1 alpha. Using oligonucleotide prim...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/0304-3940(91)90254-q
更新日期:1991-07-22 00:00:00
abstract::Intracortical facilitatory and inhibitory processes in the primary motor cortex (M1) play an important role in both the preparation and execution of motor tasks. Here we aimed to (1) confirm the existence of, and further characterise, intracortical facilitation at long conditioning-test stimulus intervals at subthresh...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2014.03.060
更新日期:2014-06-06 00:00:00
abstract::Alcohol abuse and anxiety disorders often occur concurrently, but their underlying cellular mechanisms remain unclear. N-methyl-D-aspartic acid receptors (NMDARs) have recently received attention from those interested in the neurobiology of anxiety. A chronic alcohol exposure rat model (28 consecutive days of 20% alco...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2018.09.006
更新日期:2018-11-01 00:00:00
abstract::Long-term repeated application of morphine induces plasticity changes in sensitive neurons, which represents the neurobiological basis of morphine addiction. Caveolin-1 (Cav-1) is an important scaffolding protein that plays a key role in the regulation of neurostructural plasticity. However, in-depth studies focused o...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2017.11.033
更新日期:2018-02-05 00:00:00
abstract::The somatosensory system is capable of functional reorganization following peripheral denervation or training. Studies on human amputees with phantom limb pain provided evidence that these reorganizational changes are modulated through nociceptive input. In the present study we used magnetoencephalographic recordings ...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(00)01752-3
更新日期:2001-02-09 00:00:00
abstract::Cortical spreading depression (SD) may produce some symptoms of the aura of classical migraine but it is less probable that it can account for the headache. The aversiveness of SD was examined in unanesthetized rats. In Exp. 1, rats with implanted cortical cannulae were confined in the dark compartment of the step-thr...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/0304-3940(93)90759-e
更新日期:1993-01-12 00:00:00
abstract::Alpha-2 macroglobulin (encoded by the gene A2M) is a serum pan-protease inhibitor that may be related with the pathogenesis of Alzheimer's disease (AD) because of its ability to mediate amyloid beta degradation. Recently, several groups have reported that the five-nucleotides deletion in A2M gene at the 5' splice site...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(99)00541-8
更新日期:1999-08-20 00:00:00
abstract::Alterations in the structure and physiology of the prefrontal cortex (PFC) have been found in different psychiatric disorders and some of them involve inhibitory networks, especially in schizophrenia and major depression. Changes in the structure of these networks may be mediated by the polysialylated neural cell adhe...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2012.09.032
更新日期:2012-11-14 00:00:00
abstract::To assess the neurotoxic properties of the glutamate agonist quisqualic acid (QA) in immature brain, we injected this compound (100 nmol QA/1 microliter) directly into the striatum of 7-day-old rats. QA produced neuronal necrosis and glial infiltration in 14 pups and reduced the size of the striatum and hippocampus on...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/0304-3940(86)90249-1
更新日期:1986-10-30 00:00:00
abstract::The central cardiovascular effects of neuropeptide Y (NPY) have been investigated by microinjection of the peptide into the hypothalamic paraventricular nucleus (PVN) of conscious rats. NPY (100 ng) elicited a significant bradycardia in both Long Evans (control) rats (-27 +/- beats/min) and vasopressin-deficient Bratt...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/0304-3940(88)90358-8
更新日期:1988-02-29 00:00:00
abstract::Previous studies showed that adenosine receptors of the temporal and frontal cortices were not affected in Alzheimer's disease (AD). Here, we assessed the specific binding of [3H]cyclohexyladenosine to adenosine1 (A1) receptors in hippocampus from AD subjects and age-matched controls. By both particulate membrane and ...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/0304-3940(90)90641-l
更新日期:1990-10-16 00:00:00
abstract::In ten patients with Parkinson's disease (PD) and ten age-matched healthy controls, we applied 15 30-s trains of subthreshold 5-Hz repetitive transcranial magnetic stimulation (rTMS) over the primary motor hand area. Ten minutes after rTMS, PD patients showed a significant prolongation of the transcranially evoked sil...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(00)00990-3
更新日期:2000-04-28 00:00:00
abstract::Somatostatin and several analogues were injected into rat striatum and their effects on dopamine, serotonin and their metabolites were examined at 1 h. Somatostatin induced a dose-dependent increase in dopamine turnover in the ipsilateral striatum accompanied by a reduction in turnover on the contralateral side. There...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/0304-3940(84)90034-x
更新日期:1984-02-24 00:00:00
abstract::Previous experiments have shown that arginine vasopressin (AVP) released within the septal brain area of adult male rats in response to defined stressor exposure is involved in emotionality-related behavior. We report here that a 10-min forced swimming session stimulated the release of glutamate, aspartate, arginine, ...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(99)00841-1
更新日期:1999-12-17 00:00:00
abstract::The objective of the current study was to evaluate structural changes of the corpus callosum (CC) in abstinence methamphetamine (MA) users. Shape and size of the CC in 27 MA users were compared to those of 18 healthy comparison subjects. To define the local curvature and width of the CC, medial model-based shape analy...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2005.04.082
更新日期:2005-08-12 00:00:00
abstract::Pineal gland N-acetyltransferase (NAT) activity and pineal and serum levels of melatonin declined linearly in albino rats exposed to different irradiances (low, 170 microW/cm2; moderate, 420 microW/cm2; high, 1040 microW/cm2) red light during the middle of the night. High intensity red light (1040 microW/cm2) was as e...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/0304-3940(93)90346-m
更新日期:1993-01-04 00:00:00
abstract:BACKGROUND AND OBJECTIVE:Akathisia is characterized by restlessness and crawling sensations similar to restless legs syndrome (RLS). Long latency flexor reflex (LLFR) which has helped to advance RLS pathophysiology has never been investigated in akathisia. Due to the clinical commonalities of akathisia and RLS, we inve...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2017.01.042
更新日期:2017-02-22 00:00:00
abstract::It has been shown that orexin neuropeptides contribute to morphine-induced physical dependence. The locus coeruleus (LC), which receives a dense extra-hypothalamic orexinergic projection, is a key brain region implicated in the expression of somatic signs of morphine withdrawal syndrome. The aim of the present study i...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2010.07.050
更新日期:2010-10-04 00:00:00
abstract::Presenilin-2 (PS-2) was suggested to be localized on 1q31-42 based on linkage analysis and cDNA cloning. The final identification of PS-2 as the causal gene for early-onset familial Alzheimer's disease in Voga-German pedigrees was concluded based on the point mutation found in the candidate cDNA isolated from this fam...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/s0304-3940(96)13290-0
更新日期:1997-01-17 00:00:00
abstract::Exposure to negative environmental events triggers defensive behavior and leads to the formation of aversive associative memory. Cellular and molecular changes in the central nervous system underlie this memory formation, as well as the associated behavioral changes. In general, memory process is established in distin...
journal_title:Neuroscience letters
pub_type: 杂志文章
doi:10.1016/j.neulet.2016.06.020
更新日期:2016-08-15 00:00:00