Abstract：
:Mutations of the PINK1 gene are a cause of autosomal recessive Parkinson's disease (PD). PINK1 encodes a mitochondrial kinase of unknown function which is widely expressed in both neuronal and non-neuronal cells. We have studied fibroblast cultures from four family members harbouring the homozygous p.Q456X mutation in PINK1, three of their wild-type relatives, one individual with the homozygous p.V170G mutation and five independent controls. Results showed bioenergetic abnormalities involving decreased activities of complexes I and IV along with increased activities of complexes II and III in the missense p.V170G mutant. There were increased basal levels of mitochondrial superoxide dismutase in these cells and an exaggerated increase of reduced glutathione in response to paraquat-induced free radical formation. Furthermore, swollen and enlarged mitochondria were observed in this sample. In the p.Q456X nonsense mutants, the respiratory chain enzymes were unaffected, but ATP levels were significantly decreased. These results confirm that mutations of PINK1 cause abnormal mitochondrial morphology, bioenergetic function and oxidative metabolism in human tissues but suggest that the biochemical consequences may vary between mutations.
journal_name
Exp Neuroljournal_title
Experimental neurologyauthors
Grünewald A,Gegg ME,Taanman JW,King RH,Kock N,Klein C,Schapira AHdoi
10.1016/j.expneurol.2009.05.027subject
Has Abstractpub_date
2009-09-01 00:00:00pages
266-73issue
1eissn
0014-4886issn
1090-2430pii
S0014-4886(09)00223-4journal_volume
219pub_type
杂志文章abstract：:The sciatic nerve of adult mice was transected and proximal and distal nerve stumps were sutured into a nontoxic bioresorbable nerve guide. Nerve guide lumens were either empty or filled with a gel containing 80% laminin and additional extracellular matrix components. Two weeks later cells in the L3 through L5 dorsal ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(85)90087-1
更新日期：1985-06-01 00:00:00
abstract：:In order to evaluate neurologic development, postural reflexes were tested in normal kittens from birth through 7 weeks of age until stable adult patterns of performance were attained. These reflex tests included: body and air righting, visual placing, chin placing, tactile placing, hopping, and locomotion. A protocol...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(84)90084-0
更新日期：1984-12-01 00:00:00
abstract：:Paraoxonase-2 regulates reactive oxygen species production in mitochondria. Stimulating its expression has therapeutic potential for diseases where oxidative stress plays a significant role in the pathology. Evidence suggests that the anti-diabetic drug pioglitazone may provide neuroprotection in Parkinson's disease, ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2020.113234
更新日期：2020-05-01 00:00:00
abstract：:A number of neuron-supporting functions have been ascribed to astrocytes. In this study we found that a proliferation of Purkinje cell dendritic spines, a target site for presynaptic axon terminals, was induced in cytosine arabinoside-treated cerebellar cultures by exposure to astrocyte-conditioned medium. This result...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(92)90114-6
更新日期：1992-07-01 00:00:00
abstract：:The cascade of events leading to post-traumatic epilepsy (PTE) after traumatic brain injury (TBI) remains unclear. Altered inhibition in the hippocampal formation and dentate gyrus is a hallmark of several neurological disorders, including TBI and PTE. Inhibitory synaptic signaling in the hippocampus is predominately ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2016.03.022
更新日期：2016-06-01 00:00:00
abstract：:Epidermal growth factor responsive (EGFr) neural progenitor (NP) cells have been shown to be a potential alternative tissue source for neural transplantation and for developmental study. We have shown that nonpassaged EGFr NP cells can self-renew for 2 years in neurospheres and can robustly differentiate into glia and...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.2000.7425
更新日期：2000-07-01 00:00:00
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：:Deep brain stimulation (DBS) of the subthalamic nucleus (STN) can be a highly effective treatment for Parkinson's disease (PD). However, therapeutic efficacy is limited by difficulties in consistently and correctly targeting this nucleus. Increasing evidence suggests that there is abnormal synchronization of beta freq...
journal_title：Experimental neurology
pub_type： 临床试验,杂志文章
doi：10.1016/j.expneurol.2005.11.019
更新日期：2006-03-01 00:00:00
abstract：:Focal limbic seizures often impair consciousness/awareness with major negative impact on quality of life. Recent work has shown that limbic seizures depress brainstem arousal systems, including reduced action potential firing in a key node: cholinergic neurons of the pedunculopontine tegmental nucleus (PPT). In vivo w...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2018.11.008
更新日期：2019-04-01 00:00:00
abstract：:Changes in the sleep pattern were investigated in rats given actinomycin S3 (AcS3, 0.25 mg/kg, intraventricularly), an inhibitor of RNA synthesis, at 0000, 0600, 1200, and 1800. The amounts of slow-wave (SS) and paradoxical sleep (PS) were increased during the dark period (1800 to 0600) in the groups treated at 0000, ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(83)90279-0
更新日期：1983-08-01 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
abstract：:The accumulation of damage to DNA plays a significant role in the etiology of the aging process. The importance of nutrition in delaying the aging process is well recognized. L-carnitine is a quaternary ammonium compound heterogeneously distributed in the brain. In the present study the effect of L-carnitine on DNA da...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2004.09.009
更新日期：2005-01-01 00:00:00
abstract：:Apnea of prematurity (AoP) is associated with severe and repeated episodes of arterial oxygen desaturation (intermittent hypoxia - IH), which in turn increases the number of apneas. So far, there is no data addressing whether IH leads to sex-specific respiratory consequences, neither if drugs targeting AoP are more ef...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2019.112985
更新日期：2019-10-01 00:00:00
abstract：:We examined the myelin repair potential of transplanted neural precursor cells derived from the adult human brain from tissue removed during surgery. Sections of removed brain indicated that nestin-positive cells were found predominantly in the subventricular zone around the anterior horns of the lateral ventricle and...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.2000.7539
更新日期：2001-01-01 00:00:00
abstract：:Ketamine has been reported to exert a prophylactic effect against stress-induced depressive-like behavior by modulating the guanosine-based purinergic system. However, the molecular pathways underlying its prophylactic effect and whether guanosine also elicits a similar effect remain to be determined. Here, we investi...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2020.113459
更新日期：2020-12-01 00:00:00
abstract：:Chemotherapy-related cognitive deficits are a major neurological problem, but the underlying mechanisms are unclear. The death of neural stem/precursor cell (NSC) by cisplatin has been reported as a potential cause, but this requires high doses of chemotherapeutic agents. Cisplatin is frequently used in modern oncolog...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2014.02.020
更新日期：2014-05-01 00:00:00
abstract：:The role of central noradrenergic neurons in kindled seizures was assessed by comparison of alpha 2- and beta-adrenoceptor binding in the cerebral cortex from kindled and control rats. To minimize handling, which may modify kindling-induced changes in binding, the kindling protocol involved stimulation of the amygdala...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(85)90044-5
更新日期：1985-10-01 00:00:00
abstract：:The development of reliable biological markers of nigrostriatal degeneration has important implications from both experimental and clinical viewpoints, since such biomarkers could be used for diagnostic and monitoring purposes in models of parkinsonism as well as in Parkinson's disease patients. In this study, levels ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/s0014-4886(03)00140-7
更新日期：2003-09-01 00:00:00
abstract：:The electromyographic (EMG) activity of the gastrocnemius and tibialis anterior muscles was recorded from normal geriatric subjects and from hemiplegic patients subjected to a continuous back-and-forth movement of the platform on which they stood. The platform oscillated at a fixed frequency of 0.5 Hz. Movement amplit...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(88)90156-2
更新日期：1988-02-01 00:00:00
abstract：:In the human embryo, from approximately 6 weeks gestational age (GA), dopaminergic (DA) neurons can be found in the ventral mesencephalon (VM). More specifically, the post-mitotic neurons are located in the ventral part of the tegmentum (VT), whereas no mature DA neurons are found in the neighboring dorsal part. We us...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2005.12.023
更新日期：2006-04-01 00:00:00
abstract：:Polymerized collagen rolls enclosing Schwann cells (SCs) raised in culture were grafted into cystic cavities formed after lesioning the thoracic spinal cord of adult rats. Axons were already present within the graft by 14 days after implantation and both ensheathed and myelinated axons were numerous by 28 days. This a...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(91)90043-c
更新日期：1991-11-01 00:00:00
abstract：:In the avian model of muscular dystrophy, electrophysiologic studies have shown alterations in the action potential characteristics of dystrophic muscle in vitro, supporting the notion that a membrane defect exists in avian dystrophy. As neurogenic and vascular etiologies have also been proposed, we examined the chara...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90114-7
更新日期：1986-11-01 00:00:00
abstract：:Oxaliplatin is first-line chemotherapy for colorectal cancer, but produces dose-limiting neurotoxicity. Acute neurotoxicity following infusion produces symptoms including cold-triggered fasciculations and cramps, with subsequent chronic neuropathy developing at higher cumulative doses. Axonal excitability studies were...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2010.10.002
更新日期：2011-01-01 00:00:00
abstract：:Neuroimaging genetic studies that associate genetic and epigenetic variation with neural activity or structure provide an opportunity to link genes to psychiatric disorders, often before psychopathology is discernable in behavior. Here we review neuroimaging genetics studies with participants who have Posttraumatic St...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2016.04.019
更新日期：2016-10-01 00:00:00
abstract：:The importance of mitochondria in spinal cord injury has mainly been attributed to their participation in apoptosis at the site of injury. But another aspect of mitochondrial function is the generation of more than 90% of cellular energy in the form of ATP, mediated by the oxidative phosphorylation (OxPhos) process. C...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2010.01.020
更新日期：2010-06-01 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：:Urotensin-II (U-II) is a cyclic peptide identified recently in many mammalian species including man. U-II and its receptor are expressed in the central nervous system, in the cardiovascular system and in other peripheral tissues. Although this peptide has been reported initially to be a potent vasoconstrictor, increas...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2007.12.004
更新日期：2008-04-01 00:00:00
abstract：:Twelve neurons in the median preoptic nucleus were antidromically activated by electrical stimulation of the hypothalamic paraventricular nucleus in the rat. Electrical stimulation of the subfornical organ produced excitation (N = 7) or inhibition (N = 4) of the activity of these identified units. The activity of all ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90184-6
更新日期：1986-09-01 00:00:00
abstract：:Ethanol withdrawal (ETX) in ethanol-dependent rats results in susceptibility to seizures, including generalized tonic-clonic audiogenic seizures (AGS). The inferior colliculus (IC) is strongly implicated in AGS initiation during ETX, but IC neuronal mechanisms subserving AGS are unclear. The present study examined IC ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(95)90062-4
更新日期：1995-03-01 00:00:00
abstract：:Studies of the effects of lesions of the parietal cortex suggest that this region plays a critical role in visuomotor guidance, whereas experiments on the effects of disconnecting this region from visual input have been inconsistent in supporting this hypothesis. In the present experiment, monkeys with a unilateral oc...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(83)90161-9
更新日期：1983-07-01 00:00:00
