Abstract：
:One of the most interesting findings in the field of neurodegeneration in recent years is tfche discovery of a genetic mutation in the C9orf72 gene, the most common mutation found to be causative of sporadic and familial frontotemporal lobar degeneration (FTLD), amyotrophic lateral sclerosis (ALS) and concomitant FTD-ALS (DeJesus-Hernandez et al., 2011b; Renton et al., 2011). While clinical and molecular data, such as the identification of TDP-43 being a common pathological protein (Neumann et al., 2006) have hinted at such a link for years, the identification of what was formally known as "the chromosome 9 FTLD-ALS gene" has provided a foundation for better understanding of the relationship between the two. Indeed, it is now recognized that ALS and FTLD-TDP represent a disease spectrum. In this review, we will discuss the current genetic and pathological features of the FTLD-ALS spectrum.
journal_name
Exp Neuroljournal_title
Experimental neurologyauthors
Bennion Callister J,Pickering-Brown SMdoi
10.1016/j.expneurol.2014.06.001subject
Has Abstractpub_date
2014-12-01 00:00:00pages
84-90eissn
0014-4886issn
1090-2430pii
S0014-4886(14)00188-5journal_volume
262 Pt Bpub_type
杂志文章,评审abstract：:Paired-pulse potentiation was studied in the CA1 region of rats exposed prenatally to alcohol. Pregnant rats were fed a liquid diet containing 35% ethanol-derived calories on days 3 to 21 of gestation. Control animals were fed liquid diets without ethanol. When the pups of both groups were 40 to 60 days old, hippocamp...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90203-7
更新日期：1986-08-01 00:00:00
abstract：:Neuronal migration was investigated in rodent cerebellum in vitro and in vivo. Time-lapse video recording showed that cultured neurons migrated on laminin by first extending neurites that formed contacts with other neurons. This was followed by movement of the cell nucleus inside the preformed process. No guidance cue...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(92)90119-b
更新日期：1992-08-01 00:00:00
abstract：:The loss or injury of neurons associated with oxidative and nitrosative redox stress plays an important role in the onset of various neurodegenerative diseases. Specifically, nitric oxide (NO), can affect neuronal survival through a process called S-nitrosylation, by which the NO group undergoes a redox reaction with ...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2012.06.032
更新日期：2012-11-01 00:00:00
abstract：:The basis for neuronal dysfunction following inflammatory demyelination of the central nervous system (CNS) remains poorly understood. We characterized the network response to white matter injury in the anterior visual pathway using an experimental model of optic neuritis (ON), as ON is often an early manifestation of...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2017.05.004
更新日期：2017-09-01 00:00:00
abstract：:Ghrelin has been shown to regulate neurogenesis in the hippocampus. The aim of this study was to investigate the possible influence of ghrelin on cell proliferation and neuroblast formation in the subventricular zone (SVZ) and rostral migratory system (RMS) and generation of interneurons in the olfactory bulb (OB). We...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2013.11.021
更新日期：2014-02-01 00:00:00
abstract：:It has been >20 years since studies first revealed that the brain is insulin sensitive, highlighted by the expression of insulin receptors in neurons and glia, the presence of circulating brain insulin, and even localized insulin production. Following these discoveries, evidence of decreased brain insulin receptor num...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2018.12.007
更新日期：2019-03-01 00:00:00
abstract：:The precise mechanisms underlying the efficacy of intravenous immunoglobulin (IVIg) in autoimmune neurological disorders including Guillain-Barré syndrome (GBS) are not known. Anti-ganglioside antibodies have been reported to be pathogenic in some variants of GBS, and we have developed passive transfer animal models t...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2016.05.020
更新日期：2016-08-01 00:00:00
abstract：:The left external auditory meatus was removed in 4-day-old CBA/J mice; after killing at 45 days, serial sections of the cochleae and brain stem were prepared. From these, the cross-sectional areas of spiral ganglion neurons and of 14 auditory brain stem neuronal types were measured, using a total of 210 neurons of eac...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(83)90384-9
更新日期：1983-01-01 00:00:00
abstract：:This paper focuses on transient boundaries of glia and glycoconjugates during development of the mouse central nervous system (CNS). Lectin-bound glycoconjugates, glial fibrillary acidic protein, and the J1/tenascin glycoprotein are distributed coextensively within boundaries around developing substructural arrangemen...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/s0014-4886(05)80007-x
更新日期：1990-07-01 00:00:00
abstract：:Proteoglycans have a number of important functions in the central nervous system. Aggrecan (hyaluronan-binding proteoglycan, CSPG-cs56) is found in the extracellular matrix of cartilage as well as in the developing brain. We compared the postnatal distribution of CSPG-cs56 in Long Evans (LE) and Royal College of Surge...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2012.01.023
更新日期：2012-04-01 00:00:00
abstract：:The purpose of this study was to determine whether or not tooth pulp stimulation in the rat can selectively activate the pulp nerve fibers without excitation of the periodontium and to decide if the nerve fibers situated in the pulp of the rat's incisor are involved in the nociceptive reactions caused by an intrapulpa...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90190-1
更新日期：1986-08-01 00:00:00
abstract：:Abnormal oscillatory activity in the basal ganglia is increasingly implicated in the pathophysiology of Parkinson's disease. Such activity is recorded in patients in the form of oscillations in the local field potential (LFP) picked up in the subthalamic nucleus. Previous studies have focused on correlations between f...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2012.04.024
更新日期：2012-08-01 00:00:00
abstract：:Peripheral nerves have the unique capability to regenerate after injury. Insights into regeneration of peripheral nerves after injury may have implications for neurodegenerative diseases of the nervous system. In this study, we analyzed the expression and function of desmoplakin in peripheral nerve regeneration. Desmo...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2014.12.005
更新日期：2015-02-01 00:00:00
abstract：:HIV-associated neurocognitive disorders (HAND) continue to persist despite effective control of viral replication. Although the mechanisms underlying HAND are poorly understood, recent attention has focused on altered neuronal population activity as a correlate of impaired cognition. However, while alterations in neur...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2019.113077
更新日期：2020-01-01 00:00:00
abstract：:The development of the spinal cord represents one of the most complex structure developments of the central nervous system (CNS) as it has to unfold along the longitudinal axis and within segmental cues. There it has to cope with on the one hand connection to the periphery (skeletal muscle, dermomyotome, smooth muscle...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2015.05.018
更新日期：2015-12-01 00:00:00
abstract：:This study describes the short-term effects of intracaudate microinjections of carbachol in temporarily isolated and restrained Java monkeys. The monkeys were found to display a series of motor disturbances including blepharoptosis, facial twitches, tongue protrusions, ear flattening, torticollis, and compulsive alter...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(85)90133-5
更新日期：1985-01-01 00:00:00
abstract：:One of the sexual dimorphic differences in adult rodents is neural proliferation. Here we demonstrate that physiological hormone stages can modulate this proliferation in the adult forebrain. Female mice, both pregnant and synchronized in oestrus, exhibited higher proliferating cell percentages than males in both the ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2008.12.013
更新日期：2009-04-01 00:00:00
abstract：:The majority of human spinal cord injuries involve gray matter loss from the cervical or lumbar enlargements. However, the deficits that arise from gray matter damage are largely masked by the severe deficits due to associated white matter damage. We have developed a model to examine gray matter-specific deficits and ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1999.7016
更新日期：1999-03-01 00:00:00
abstract：:Spinal axons in the larval sea lamprey regenerate following a complete spinal transection. It is not known whether regenerating growth cones require contact with cellular elements or whether the basement membrane and collagenous meninx primitiva which surround the spinal cord are sufficient for neurite out-growth. To ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(91)90114-r
更新日期：1991-04-01 00:00:00
abstract：:Previous studies have shown that the herpes simplex virus type 2 protein kinase ICP10 PK activates the Ras/MEK/MAPK pathway in nonneuronal cells. Here we report that ectopically expressed ICP10 PK has anti-apoptotic activity in various paradigms of neuronal cell death. Neuronally differentiated PC12 cells and primary ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.2001.7849
更新日期：2002-03-01 00:00:00
abstract：:Recent studies have implicated nitric oxide (NO) in several mechanisms related to neuronal degeneration and synaptic plasticity. In the present study, two models of traumatic neuronal injury were used to examine the expression of NOS following neuronal injury and its relationship to axonal sprouting and neuronal degen...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1993.1050
更新日期：1993-04-01 00:00:00
abstract：:Dopaminergic (DAergic) hypofunction in the medial prefrontal cortex (mPFC) has been implicated in the negative and cognitive symptoms of schizophrenia and is regulated by serotonergic (5-HTergic) neurons in the dorsal raphe nucleus (DRN). The lateral habenula (LHb) is a key element in controlling DRN 5-HT neurons. We ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2019.05.005
更新日期：2019-08-01 00:00:00
abstract：:The recently discovered endocannabinoid system (ECS), which includes endocannabinoids and the proteins that metabolize and bind them, has been implicated in multiple regulatory functions both in health and disease. Several studies have suggested that ECS is centrally and peripherally involved in the processing of pain...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2010.03.029
更新日期：2010-07-01 00:00:00
abstract：:This paper reports that endolymphatic hydrops causes a significant reduction of retrogradely labeled cell bodies of the ipsilateral trigeminal ganglion following application of horseradish peroxidase in the cochlea. We previously showed that the trigeminal ganglion is a source of primary sensory innervation to the coc...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1998.6813
更新日期：1998-06-01 00:00:00
abstract：:We studied electrophysiological and morphological properties of the Aalpha- and Abeta-fibers in the regenerating sciatic nerve to establish whether these fiber types regenerate in numerical proportion and whether and how the electrophysiological properties of these fiber types are adjusted during regeneration. Compoun...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2004.01.019
更新日期：2004-06-01 00:00:00
abstract：:Spinal cord injury (SCI) is a tragic event causing irreversible losses of sensory, motor, and autonomic functions, that may also be associated with chronic neuropathic pain. Serotonin (5-HT) neurotransmission in the spinal cord is critical for modulating sensory, motor, and autonomic functions. Following SCI, 5-HT axo...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2019.05.007
更新日期：2019-08-01 00:00:00
abstract：:Embryonic substantia nigra grafts partially reinnervate the dopamine-denervated corpus striatum when implanted adjacent to that structure. This reinnervation is generally limited to a small portion of the denervated striatum and does not completely compensate for the behavioral effects of a 6-hydroxydopamine lesion of...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(87)90151-8
更新日期：1987-02-01 00:00:00
abstract：:Huntington's disease is an autosomal dominant neurogenetic disorder leading to striatal atrophy, characterized by involuntary movements. Voluntary movements also deteriorate, but the neurophysiological mechanisms are less understood. We investigated voluntary movement execution and its neural correlates by means of mo...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2008.11.018
更新日期：2009-03-01 00:00:00
abstract：:A 168-nucleotide exon, found in alternatively spliced amyloid precursor protein (APP) mRNAs, encodes a Kunitz protease inhibitor (KPI) domain. Kainic acid (ip) caused a selective increase of KPI mRNA in rat hippocampus. By in situ hybridization, KPI mRNA was induced in the neuronal layers of the hippocampus 11-12 h af...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(92)90191-r
更新日期：1992-12-01 00:00:00
abstract：:Transplantation of genetically engineered cells can provide sustained focal delivery of naturally occurring molecules, including neurotransmitters and growth factors. We have engineered immortalized mouse cortical neurons and glia to deliver GABA by driving GAD(65) expression. Engineered cell lines showed GAD(65) mRNA...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1999.7305
更新日期：2000-02-01 00:00:00
