Abstract：
:Successive fusion events between transport vesicles and their target membranes mediate trafficking of secreted, membrane- and organelle-localised proteins. During the initial steps of this process, termed the secretory pathway, COPII vesicles bud from the endoplasmic reticulum (ER) and fuse with the cis-Golgi membrane, thus depositing their cargo. This fusion step is driven by a quartet of SNARE proteins that includes the cis-Golgi t-SNARE Membrin, encoded by the GOSR2 gene. Mis-sense mutations in GOSR2 result in Progressive Myoclonus Epilepsy (PME), a severe neurological disorder characterised by ataxia, myoclonus and seizures in the absence of significant cognitive impairment. However, given the ubiquitous and essential function of ER-to-Golgi transport, why GOSR2 mutations cause neurological dysfunction and not lethality or a broader range of developmental defects has remained an enigma. Here we highlight new work that has shed light on this issue and incorporate insights into canonical and non-canonical secretory trafficking pathways in neurons to speculate as to the cellular and molecular mechanisms underlying GOSR2 PME. This article is part of a Special Issue entitled: SNARE proteins: a long journey of science in brain physiology and pathology: from molecular.
journal_name
Neurosciencejournal_title
Neuroscienceauthors
Jepson JEC,Praschberger R,Krishnakumar SSdoi
10.1016/j.neuroscience.2019.03.057subject
Has Abstractpub_date
2019-11-10 00:00:00pages
41-49eissn
0306-4522issn
1873-7544pii
S0306-4522(19)30219-2journal_volume
420pub_type
杂志文章,评审相关文献
NEUROSCIENCE文献大全abstract：:The aim of this study was to examine the development of the basalocortical pathway by using choline acetyltransferase and nerve growth factor receptor immunocytochemistry, acetylcholinesterase histochemistry and retrograde axonal transport. The observations were made in the ferret because in this species brain develop...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/0306-4522(91)90056-t
更新日期：1991-01-01 00:00:00
abstract：:Chronic administration of mu-opioid receptor agonists is known to produce adaptive changes within noradrenergic neurons of the locus coeruleus. Although mu-opioid receptors are densely expressed by locus coeruleus neurons, the effects of acute and chronic administration of agonists on the subcellular distribution of m...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/s0306-4522(01)00426-2
更新日期：2001-01-01 00:00:00
abstract：:Interleukin-1 (IL-1) plays a crucial role in stress responses and its mRNA is induced in the brain by stress load; however, the precise role of IL-1 in higher brain functions and their abnormalities is largely unknown. Here, we report that IL-1 receptor antagonist (IL-1Ra) knockout (KO) mice, which lack IL-1Ra molecul...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2011.06.031
更新日期：2011-09-29 00:00:00
abstract：:Glutamate is the most abundant excitatory neurotransmitter in the vertebrate central nervous system. It is widely assumed that neurons using this transmitter derive it from several sources: (i) synthesizing it themselves from alpha-ketoglutarate or aspartate, (ii) synthesize it from glial-derived glutamine, or (iii) t...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/0306-4522(94)90249-6
更新日期：1994-05-01 00:00:00
abstract：:The p38 member of the mitogen-activated protein kinase superfamily is engaged by phosphorylation in response to environmental stress signals, and may have either permissive or inhibitor roles upon cell proliferation. The cell cycle in the proliferative zone of the retina is tightly controlled and proceeds in synchrony...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/s0306-4522(02)00096-9
更新日期：2002-01-01 00:00:00
abstract：:Probenecid has been used for decades to treat gout, and recent studies have revealed it is also a specific inhibitor of the pannexin-1 channel. It has been reported that the pannexin-1 channel is involved in ischemic injury. Here, we investigated the neuroprotective effect and the possible mechanisms of action of prob...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2015.05.070
更新日期：2015-08-20 00:00:00
abstract：:We examined the expression of SIRT1 in several experimental paradigms of human pathologies. We used a neuroblastoma cell line (B65), neuronal primary cultures (hippocampus and cerebellar granule cells) and in vivo approaches in rat and senescence murine models (SAM). Cell cultures and rats were treated with several we...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2008.04.065
更新日期：2008-07-17 00:00:00
abstract：:Dopamine (DA) axons and receptors have recently been identified in the primate thalamus, including the mediodorsal thalamic nucleus (MD). In order to determine whether the DA innervation of the primate MD shares the anatomical features of the mesocortical or nigrostriatal DA projections, we performed tract-tracing and...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2006.08.056
更新日期：2006-12-28 00:00:00
abstract：:It is well established that chronic food restriction enhances sensitivity to the rewarding and motor-activating effects of abused drugs. However, neuroadaptations underlying these behavioral effects have not been characterized. The purpose of the present study was to explore the possibility that food restriction produ...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/s0306-4522(03)00227-6
更新日期：2003-01-01 00:00:00
abstract：:In several neurological disorders including hyperhomocysteinemia, homocysteine (Hcy) accumulates in the brain, and acts as a potent neurotoxin. However, the molecular mechanisms induced by increased levels of Hcy in brain are not well understood. Here we show an activation of the extracellular signal-regulated kinases...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2005.03.034
更新日期：2005-01-01 00:00:00
abstract：:Cultured neurons offer many advantages over a slice preparation for whole-cell patch-clamp studies, such as better control over the environment and space clamp control. However, heterogeneous cultures of neurons present problems in distinguishing the cell type from which recordings are made. The present study uses cor...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/s0306-4522(99)00153-0
更新日期：1999-01-01 00:00:00
abstract：:The functional changes of astrocytes are deeply involved in neurodegenerating processes of various CNS diseases. ATP is released during various neuronal damages such as brain ischemia and may control astrocyte functions. We examined the effect of ATP on the production of nitric oxide in the cultured astrocytes from ra...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/s0306-4522(02)00804-7
更新日期：2003-01-01 00:00:00
abstract：:The experiments described herein present a method for tracking diffusion of the glucocorticoid receptor agonist RU28362 in brain following i.c.v. drug administration. A useful property of glucocorticoid receptor is that it is primarily cytoplasmic when unbound and rapidly translocates to the nucleus when bound by liga...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2006.04.067
更新日期：2006-09-01 00:00:00
abstract：:Physiological and anatomical investigations are commonly combined in experimental models. When studying the lower urinary tract (LUT), it is often of interest to perform both urodynamic studies and retrogradely labeled neurons innervating the peripheral target organs. However, it is not known whether the use of anatom...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2009.11.037
更新日期：2010-03-10 00:00:00
abstract：:After experimentally inducing long term changes in transmitter release, a series of frog neuromuscular junctions were studied with intracellular recording and then semi-serially sectioned and examined in the electron microscope. Transmitter release per unit length of motor nerve terminal was well correlated with sever...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/0306-4522(85)90187-3
更新日期：1985-11-01 00:00:00
abstract：:Maternal separation (MS) during the first postnatal weeks induces alcohol intake and a reduction in the expression of glucocorticoid receptors (GR). Adults' alcohol consumption may depend on changes in the endocannabinoid system (eCBs). Our goal was to evaluate the status of the eCBs before the exposition to alcohol t...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2012.07.071
更新日期：2012-10-25 00:00:00
abstract：:The possible involvement of fibroblast growth factor receptor (FGFR) activation in the dorsal root ganglion (DRG) was examined following peripheral nerve injury in the rat. Ligation of the sciatic nerve down-regulated FGFR2, -3 and -4 mRNA; however, the expression of FGFR1 mRNA showed no change. Activation of FGFR was...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2007.08.024
更新日期：2007-11-30 00:00:00
abstract：:The postural, ocular motor, perceptive and neurovegetative syndromes resulting from unilateral vestibular neurectomy (UVN) symptoms could generate a stress and thereby activate the hypothalamo-pituitary-adrenal (HPA) axis. This study was aimed at determining whether UVN causes changes in the activity of the HPA axis, ...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2009.02.070
更新日期：2009-06-02 00:00:00
abstract：:Compulsive drug abuse has been conceptualized as a behavioral state where behavioral stimuli override normal decision making. Clinical studies of methamphetamine users have detailed decision making changes and imaging studies have found altered metabolism and activation in the parietal cortex. To examine the molecular...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2006.08.076
更新日期：2007-01-05 00:00:00
abstract：:Decreased levels of soluble ubiquitin carboxy-terminal hydrolase L1 (UCHL1) have been reported in the brains of sporadic Alzheimer's disease (AD) patients, and the introduction of UCHL1 rescued the synaptic and cognitive function of AD model mice. Obviously, a reduction in the levels of UCHL1 may play a role in the pa...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2014.06.013
更新日期：2014-09-05 00:00:00
abstract：:Relay cells of dorsal lateral geniculate nucleus (LGN) receive a Class 1 glutamatergic input from the retina and a Class 2 input from cortical layer 6. Among the properties of Class 2 synapses is the ability to activate metabotropic glutamate receptors (mGluRs), and mGluR activation is known to affect thalamocortical ...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2013.03.043
更新日期：2013-07-09 00:00:00
abstract：:Central blockade of mineralocorticoid receptors (MRs) or angiotensin II type 1 receptors (AT1Rs) attenuates aldosterone (aldo)-salt induced hypertension. We examined the role of the subfornical organ (SFO), aldo synthesized locally in the brain, and MR and AT1R specifically in the paraventricular nucleus (PVN) in aldo...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2015.11.055
更新日期：2016-02-09 00:00:00
abstract：:By employing both in vivo and in vitro labeling techniques, opiate receptors were labeled with tritiated diprenorphine in the monkey brain and localized by light microscopic autoradiography. Both methods of labeling gave similar results, allowing a description of discrete areas having opiate binding sites. High concen...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/0306-4522(82)90066-5
更新日期：1982-03-01 00:00:00
abstract：:We have shown that synaptosomal membrane Na+, K+-ATPase activity is stimulated or inhibited by norepinephrine according to the presence or absence of a brain soluble fraction. Gel filtration of such soluble fraction has allowed the separation of two fractions, peaks I and II, able to stimulate and inhibit Na+, K+-ATPa...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/s0306-4522(98)00421-7
更新日期：1999-05-01 00:00:00
abstract：:Recent findings indicate that neurosteroids could act as important keys during the brain development. Fluctuations in neonatal allopregnanolone (AlloP) could result in altered pharmacological properties of the GABA(A) receptor system in adulthood. Recent studies demonstrated that neurosteroids play a critical role in ...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2008.04.062
更新日期：2008-07-17 00:00:00
abstract：:Multiple sclerosis (MS) is an autoimmune disease in which more than 70% of patients experience visual disturbance as the earliest symptoms. Lysolecithin (LPC)-induced focal demyelination model has been developed to evaluate the effects of different therapies on myelin repair improvement. In this study, the effects of ...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2016.12.037
更新日期：2017-03-06 00:00:00
abstract：:The effect of capsaicin on voltage-activated calcium currents was investigated in voltage-clamped somata of cultured adult rat dorsal root ganglion neurons. About half the neurons studied were sensitive to capsaicin, which induced an inward current at negative membrane potentials accompanied by an increase in membrane...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/0306-4522(91)90137-d
更新日期：1991-01-01 00:00:00
abstract：:Microglia, the innate immune cells of the brain, regulate brain development through many processes such as synaptic pruning, supporting cell genesis and phagocytosing living and dying cells. There are sex differences in these same developmental processes throughout the brain, thus microglia may contribute to brain sex...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2020.10.009
更新日期：2021-01-15 00:00:00
abstract：:Previous studies have raised the possibility that a decrease in voltage-gated K+ currents may contribute to hyperexcitability of injured dorsal root ganglion (DRG) neurons and the emergence of neuropathic pain. We examined the effects of axotomy on mRNA levels for various Kv1 family subunits and voltage-gated K+ curre...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2003.11.014
更新日期：2004-01-01 00:00:00
abstract：:Microglia are the prime cellular sources of reactive oxygen species (ROS) in the central nervous system (CNS). Chronic activation of microglia has been linked to aging-associated neurodegenerative diseases such as Alzheimer's disease (AD) and Parkinson's disease (PD) since they produce excessive amounts of ROS for a p...
journal_title：Neuroscience
pub_type： 杂志文章
doi：10.1016/j.neuroscience.2015.10.048
更新日期：2015-12-17 00:00:00
