Abstract：
:Oxidative stress and protein aggregation are biochemical hallmarks of Parkinson's disease (PD), a frequent sporadic late-onset degenerative disorder particularly of dopaminergic neurons in the substantia nigra, resulting in impaired spontaneous movement. PARK6 is a rare autosomal-recessively inherited disorder, mimicking the clinical picture of PD with earlier onset and slower progression. Genetic data demonstrated PARK6 to be caused by mutations in the protein PINK1, which is localized to mitochondria and has a serine-threonine kinase domain. To study the effect of PINK1 mutations on oxidative stress, we used primary fibroblasts and immortalized lymphoblasts from three patients homozygous for G309D-PINK1. Oxidative stress was evident from increases in lipid peroxidation and in antioxidant defenses by mitochondrial superoxide dismutase and glutathione. Elevated levels of glutathione reductase and glutathione-S-transferase were also observed. As a putative cause of oxidation, a mild decrease in complex I activity and a trend to superoxide elevation were detectable. These data indicate that PINK1 function is critical to prevent oxidative damage and that peripheral cells may be useful for studies of progression and therapy of PARK6.
journal_name
Neurobiol Disjournal_title
Neurobiology of diseaseauthors
Hoepken HH,Gispert S,Morales B,Wingerter O,Del Turco D,Mülsch A,Nussbaum RL,Müller K,Dröse S,Brandt U,Deller T,Wirth B,Kudin AP,Kunz WS,Auburger Gdoi
10.1016/j.nbd.2006.10.007subject
Has Abstractpub_date
2007-02-01 00:00:00pages
401-11issue
2eissn
0969-9961issn
1095-953Xpii
S0969-9961(06)00258-0journal_volume
25pub_type
杂志文章abstract：:Neuropeptide Y (NPY) is an important 36 amino acid peptide that is abundantly expressed in the mammalian CNS and is known to be an endogenous modulator of seizure activity, including in rat models of Genetic Generalised Epilepsy (GGE) with absence seizures. Studies have shown that viral-mediated "gene therapy" with ov...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2018.01.016
更新日期：2018-05-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2015.02.031
更新日期：2015-06-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2016.03.013
更新日期：2016-07-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
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更新日期：2006-04-01 00:00:00
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journal_title：Neurobiology of disease
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更新日期：2000-08-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章,评审
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更新日期：2014-12-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2005.03.003
更新日期：2005-11-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1006/nbdi.2000.0314
更新日期：2000-12-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2012.06.016
更新日期：2012-10-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2010.01.015
更新日期：2010-05-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2006.08.006
更新日期：2006-12-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2005.08.017
更新日期：2006-03-01 00:00:00
abstract：:Amyloid-β is a peptide released by synapses in physiological conditions and its pathological accumulation in brain structures necessary for memory processing represents a key toxic hallmark underlying Alzheimer's disease. The oligomeric form of Amyloid-β (Aβο) is now believed to represent the main Amyloid-β species af...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2018.07.001
更新日期：2018-10-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2020.104821
更新日期：2020-06-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2018.02.014
更新日期：2018-06-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章,评审
doi：10.1016/j.nbd.2020.105088
更新日期：2020-12-01 00:00:00
abstract：:Mesenchymal stem cells (MSCs) are reported to possess immunomodulatory properties. Previous reports have demonstrated the beneficial effects of MSC-transplantation in focal cerebral ischemia animal models. In this study, we have investigated the neuroimmunomodulatory functions of human MSCs, transplanted in a rat foca...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2010.12.009
更新日期：2011-03-01 00:00:00
abstract：:Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disease characterized by the progressive loss of motor neurons, leading to profound weakness and eventual death of affected individuals. For the vast majority of patients with ALS, the etiology of the disorder is unknown, and although multiple clinical...
journal_title：Neurobiology of disease
pub_type： 杂志文章,评审
doi：10.1006/nbdi.1999.0266
更新日期：1999-10-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2019.04.004
更新日期：2019-07-01 00:00:00
abstract：:Prion strains yield specific neuropathological features including spongiform degeneration and deposition patterns of pathological prion protein. Their invariant regional distribution, following variations in the infection route, has led to the proposal that prions replicate preferentially in defined neuro-anatomical a...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2010.09.010
更新日期：2011-01-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1006/nbdi.2001.0399
更新日期：2001-08-01 00:00:00
abstract：:Excitotoxicity plays a major role in the pathogenesis of Huntington disease (HD), a fatal neurodegenerative disorder. Adenosine A(2A) receptors (A(2A)Rs) modulate excitotoxicity and have been suggested to play a pathogenetic role in HD. The main aim of this study was to evaluate the effect of A(2A)R blockade on the ex...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2009.09.012
更新日期：2010-01-01 00:00:00
abstract：:In addition to the substantial biological diversity among humans, our limited ability to reliably measure expression changes of small magnitude significantly reduces our capacity to obtain convergent sets of transcriptome data in postmortem brain. In particular, differences in the structure and sensitivity/reproducibi...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2004.10.020
更新日期：2005-04-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2005.11.006
更新日期：2006-05-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2018.04.006
更新日期：2018-07-01 00:00:00
abstract：:Thermal hyperpnea, a pattern of breathing during hyperthermia that is characterized by an increase in tidal volume as well as breathing frequency, is known to lead to respiratory alkalosis. Thermal hyperpnea-induced respiratory alkalosis is linked to febrile seizures (FS). The heat-sensitive transient receptor potenti...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2018.08.004
更新日期：2018-11-01 00:00:00
abstract：:Recent studies indicate that the Parkinson's disease-linked leucine-rich repeat kinase 2 (LRRK2) modulates cytoskeletal functions by regulating actin and tubulin dynamics, thereby affecting neurite outgrowth. By interactome analysis we demonstrate that the binding of LRRK2 to tubulins is significantly enhanced by phar...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2012.12.019
更新日期：2013-06-01 00:00:00
abstract：:The hyh mouse carrying a point mutation in the gene encoding for soluble N-ethylmaleimide-sensitive factor (NSF) attachment protein alpha (alpha-SNAP) develops inherited hydrocephalus. The investigation was designed to study: (i) the clinical evolution of hyh mice; (ii) factors other than the alpha-SNAP mutation that ...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2006.02.009
更新日期：2006-07-01 00:00:00
abstract：:It is widely accepted that the loss of function of different cellular proteins following their aggregation into highly stable aggregates or the gain of pathologic function of the resulting macromolecular assemblies or both processes are tightly associated to distinct debilitating neurodegenerative diseases such as Alz...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2017.03.011
更新日期：2018-01-01 00:00:00
abstract：:The developing rodent brain is vulnerable to pharmacological blockade of N-methyl-d-aspartate (NMDA) receptors which can lead to severe and disseminated apoptotic neurodegeneration. Here, we show that systemic administration of the NMDA receptor antagonist MK801 to 7-day-old rats leads to impaired activity of extracel...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2004.03.013
更新日期：2004-07-01 00:00:00
