Abstract：
:We compared mitochondrial respiratory chain function, mitochondrial DNA (mtDNA) integrity, and oxidative stress levels in muscle, myoblasts, fibroblasts and cybrids, from 12 amyotrophic lateral sclerosis (ALS) patients with 28 control samples. Mitochondrial respiratory chain enzyme activities were normal in muscle, myoblast and fibroblast cultures from ALS patients, as were levels of mtDNA in muscle. Rearranged muscle mtDNA species were not detected by Southern blot hybridization in any of the samples and no difference was found in the number of deleted mtDNA species detected by long-range PCR. Platelet-derived cybrid studies confirmed the absence of a systemic mtDNA abnormality. Aconitase activity measurements did not indicate increased oxidative damage in muscle tissue, or in myoblasts or fibroblasts from ALS patients cultured under basal conditions. We did, however, find an increased sensitivity to oxidative stress in myoblasts from ALS patients exposed to paraquat. This altered sensitivity appears to be due to a nuclear rather than a mtDNA abnormality. Motor neurons have a large relative size and metabolic activity, and would be expected to be exposed to a greater degree of oxidative stress than most tissues throughout life. In addition, neurons are postmitotic cells, with poor regenerative potential. We do not have a ready method to study this in neural tissue of living patients, but the oxidative stress identified in myoblasts would translate into oxidative damage more readily in motor neurons than in other tissues.
journal_name
Exp Neuroljournal_title
Experimental neurologyauthors
Bradley LJ,Taanman JW,Kallis C,Orrell RWdoi
10.1016/j.expneurol.2009.04.007subject
Has Abstractpub_date
2009-07-01 00:00:00pages
92-7issue
1eissn
0014-4886issn
1090-2430pii
S0014-4886(09)00143-5journal_volume
218pub_type
杂志文章abstract：:Ghrelin, a 28-amino acid peptide, is an endogenous ligand for the growth hormone secretagogue (GHS) receptor. Our previous data showed that ghrelin could inhibit apoptosis in Parkinson's disease (PD) models both in vitro and in vivo. There is now growing evidence that oxidative stress has a critical role in the etiolo...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2009.11.009
更新日期：2010-03-01 00:00:00
abstract：:The piriform cortex is recognized to play critical roles in focal ictogenesis, both in animal models and in humans. We review here the contribution of in vitro studies performed on rodent brain tissue that were aimed at understanding the ictogenic properties of the piriform cortex and the contiguous olfactory areas. D...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2019.113014
更新日期：2019-11-01 00:00:00
abstract：:In previous studies on sensory axons we reported that the effect of a conditioning lesion on increasing regeneration rate was enhanced if the two lesions were superimposed, rather than made at separate sites on the nerve, and proposed that this was due to the growth of axons through nerve predegenerated by the conditi...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(85)90027-5
更新日期：1985-11-01 00:00:00
abstract：:Previous two-dimensional (2-D) gel electrophoretic studies of proteins secreted by degenerating mammalian peripheral nerves (Ignatius et al., 1986, Proc. Natl. Acad. Sci. USA 83: 1125-1129; Muller et al., 1986, J. Cell Biol. 102: 393-402) detected the up-regulation of two proteins of 67-70 and 34-37 kDa, although they...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1999.7332
更新日期：2000-03-01 00:00:00
abstract：:Rats were injected with progressively increasing doses of morphine or meperidine during a period of 3 to 40 days. From this colony of animals individual rats were used at 3- to 4-day intervals for electrophysiologic experiments to analyze the activity of nociceptive neurons in the somesthetic thalamus. After an i.p. i...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(84)90051-7
更新日期：1984-01-01 00:00:00
abstract：:The human small heat shock proteins (HSPBs) form a family of molecular chaperones comprising ten members (HSPB1-HSPB10), whose functions span from protein quality control to cytoskeletal dynamics and cell death control. Mutations in HSPBs can lead to human disease and particularly point mutations in HSPB1 and HSPB8 ar...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2016.08.014
更新日期：2016-12-01 00:00:00
abstract：:Despite the importance of the periaqueductal gray (PAG) in the modulation of nociception and pain, many aspects of the roles of the different columns of the PAG in descending controls: facilitation and inhibition, are not understood. Employing a tonic muscle pain model established by i.m. injection of 5.8% saline into...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2014.04.019
更新日期：2014-07-01 00:00:00
abstract：:The aim of this study was to characterize the microglial and astroglial reactions to degeneration of (a) hippocampal CA1 pyramidal cells and dentate hilar neurons induced by cerebral ischemia and (b) CA3 pyramidal cells and dentate hilar neurons induced by intraventricular injections of kainic acid (KA). The microglia...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1993.1041
更新日期：1993-03-01 00:00:00
abstract：:Electrical stimulation of the midbrain tegmentum can produce differential modulation of quiet biting attack and affective defense behavior elicited from the hypothalamus of the cat. Stimulation of the lateral half of the tegmentum facilitated quiet biting attack and suppressed affective defense. Conversely, stimulatio...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/S0014-4886(84)90112-2
更新日期：1984-02-01 00:00:00
abstract：:Mutations in the human NOTCH3 gene cause cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), but the pathogenesis of CADASIL has remained unclear. Recently, endocytosis of the Notch ectodermal domain into ligand-expressing cells, called transendocytosis, has come to be...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2011.10.020
更新日期：2012-01-01 00:00:00
abstract：:Graft-induced dyskinesia has emerged as a problematic side effect after transplantation of fetal dopamine cells into the striatum of patients with Parkinson's disease. These adverse effects of dystonic and choreatiform hyperkinesias that persisted even after withdrawal of L-DOPA medication are not yet fully understood...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2011.09.017
更新日期：2011-12-01 00:00:00
abstract：:Excitant amino acids are implicated in audiogenic seizure (AGS) susceptibility in the genetically epilepsy-prone rat (GEPR). In the present study systemic administration of NMDA receptor antagonists significantly decreased AGS severity in the GEPR. Systemic administration of the competitive NMDA antagonists 3-((+-)-2-...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1993.1021
更新日期：1993-02-01 00:00:00
abstract：:This study characterizes the impact of increasing-intensity treadmill exercise (iTR) on noradrenergic (NE) and serotonergic (5HT) modulation of neuropathic pain. Following sciatic nerve transection and repair (SNTR) rats developed significant mechanical and thermal hyperalgesia that was partially prevented by iTR perf...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2017.10.007
更新日期：2018-01-01 00:00:00
abstract：:Breathing is a life-sustaining behavior that in mammals is accomplished by activation of dedicated muscles responsible for inspiratory and expiratory forces acting on the lung and chest wall. Motor control is exerted by specialized pools of motoneurons in the medulla and spinal cord innervated by projections from mult...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2016.09.014
更新日期：2017-01-01 00:00:00
abstract：:The purpose of this study was to assess the respiratory and tonic activity of the abdominal muscles and the postinspiratory activity of the diaphragm (stage 1 expiration) in rats during sleep while they breathed air, hypercapnic, and hypoxic gas mixtures. ECoG and neck EMG recordings enabled the differentiation to be ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(88)90064-7
更新日期：1988-07-01 00:00:00
abstract：:There is a consensus that active sleep (AS; i.e., REM sleep) is produced by cholinergic projections from the pedunculopontine tegmental nuclei (PPT) that activate AS-on neurons in the nucleus pontis oralis (NPO) that are components of the AS-Generator. However, there is a growing body of evidence indicating that other...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2012.08.001
更新日期：2012-11-01 00:00:00
abstract：:The therapeutic effect of glial progenitor transplantation in diseases of dysmyelination is currently attributed to the formation of new myelin. Using magnetic resonance imaging (MRI), we show that the therapeutic outcome in dysmyelinated shiverer mice is dependent on the extent of cell migration but not the presence ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2017.02.005
更新日期：2017-05-01 00:00:00
abstract：:C2 spinal cord hemisection results in synaptic and astroglial changes in the phrenic nucleus which have been associated with the recovery of the ipsilateral hemidiaphragm during expression of the crossed phrenic phenomenon. As part of our ongoing analysis of the neurotransmitters involved, the present study investigat...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1999.7238
更新日期：1999-12-01 00:00:00
abstract：:Historically, the immune response after spinal cord injury was considered largely detrimental owing to the release of neurotoxic factors. While there is validity to this view, there is much greater heterogeneity of immune cells than was previously realized. Associated with this heterogeneity of immune cell subtypes, t...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2014.01.025
更新日期：2014-08-01 00:00:00
abstract：:The antiretroviral toxic neuropathy, a distal sensory polyneuropathy associated with antiretroviral treatment, is a frequently occurring neurological complication during treatment of patients with AIDS and often leads to discontinuation of antiretroviral therapy. The mechanisms by which antiretroviral drugs contribute...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2015.02.036
更新日期：2015-05-01 00:00:00
abstract：:Exposure of the rodent striatum to quinolinic acid (QA, N-methyl-D-aspartate receptor agonist) induces immediate early gene (IEG; c-fos, c-jun, jun-B, zif/268) expression that may extend 12-24 h after injection. In order to determine the specificity of the prolonged IEG response to the QA injection, the temporal patte...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1997.6427
更新日期：1997-04-01 00:00:00
abstract：:Although prior studies have implicated maladaptive remodeling of dendritic spines on wide-dynamic range dorsal horn neurons as a contributor to pain after spinal cord injury, there have been no studies on dendritic spines after peripheral nerve injury. To determine whether dendritic spine remodeling contributes to neu...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2011.08.028
更新日期：2011-12-01 00:00:00
abstract：:We investigated the role of the NO/cGMP system in the vasodilatory response to hypercapnia after cortical spreading depression (CSD) in barbiturate anesthetized rats in vivo. Regional cerebral blood flow (rCBF) was measured by laser Doppler flowmetry (LDF). Hypercapnia (arterial pCO2 50-60 mm Hg) increased rCBF by 2.8...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2006.07.007
更新日期：2006-12-01 00:00:00
abstract：:The effects of sex differences and hormonal factors on the locomotor alterations following intrastriatal injections of quinolinic acid (QA) and the ability of fetal striatal transplants to reverse those effects were examined. Male, female, or ovariectomized female rats received bilateral injections of 150 nmol QA or v...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(91)90105-l
更新日期：1991-03-01 00:00:00
abstract：:When adrenal medullary cells are cultured in vitro, tyrosine hydroxylase (TH) mRNA, preproenkephalin (PPEnk) mRNA, and methionine enkephalin (Mek) immunoreactivity was markedly increased compared with intact adrenal medullary cells in situ, suggesting an increased biosynthesis of catecholamines and enkephalin-containi...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1999.7267
更新日期：2000-01-01 00:00:00
abstract：:Dopamine (DA) release and metabolism within the substantia nigra (SN) were studied in normal rats, rats with unilateral 6-hydroxydopamine (6-OHDA) lesions, and 6-OHDA-lesioned rats treated with glial cells line-derived neurotrophic factor (GDNF). Animals with > 99% DA depletions, as determined by apomorphine-induced c...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1997.6571
更新日期：1997-09-01 00:00:00
abstract：:The control of gene expression by miRNAs has been widely investigated in different species and cell types. Following a probabilistic rather than a deterministic regimen, the action of these short nucleotide sequences on specific genes depends on intracellular concentration, which in turn reflects the balance between b...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2013.07.022
更新日期：2013-10-01 00:00:00
abstract：:Silent strokes occur more frequently than classic strokes; however, symptoms may go unreported in spite of lasting tissue damage. A silent stroke may indicate elevated susceptibility to recurrent stroke, which may eventually result in apparent and lasting impairments. Here we investigated if multiple silent strokes to...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2012.05.007
更新日期：2012-08-01 00:00:00
abstract：:As early as 1928, Cajal suggested that plaques contain a trophic substance which attracts neurites. Recently, basic fibroblast growth factor (bFGF) levels were shown to be elevated in Alzheimer's disease (AD) and localized to plaques and neurofibrillary tangles. We sought to clarify the subtype of plaques which contai...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1993.1202
更新日期：1993-12-01 00:00:00
abstract：:The conditioned corral preference paradigm was used to assess reinforcing effects of substance P (SP) and its N- and C-terminal fragments injected unilaterally into the region of the nucleus basalis magnocellularis (NBM) in rats. Behavioral testing was carried out in a circular open field, consisting of 4 quadrants eq...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(92)90063-v
更新日期：1992-02-01 00:00:00
