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Specialty: Neurology
Condition: Mitochondrial Disease

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Total 22 results found since Jan 2013.

Upregulated hexokinase 2 expression induces the apoptosis of dopaminergic neurons by promoting lactate production in Parkinson's disease
In this study, we examined the expression of hexokinase 2 (HK2) and lactate dehydrogenase (LDH), the key enzymes in glycolysis, and lactate levels in the substantia nigra pars compacta (SNpc) of a MPTP-induced mouse model of PD and in MPP+-treated SH-SY5Y cells. We found that the expression of HK2 and LDHA and the lactate levels were markedly increased in the SNpc of MPTP-treated mice and in MPP+-treated SH-SY5Y cells. Exogenous lactate treatment led to the apoptosis of SH-SY5Y cells. Intriguingly, lactate production and the apoptosis of dopaminergic neurons were suppressed by the application of 3-bromopyruvic acid (3-Brpa...
Source: Neurobiology of Disease - January 1, 2022 Category: Neurology Authors: Jingyi Li Longmin Chen Qixiong Qin Danlei Wang Jingwei Zhao Hongling Gao Xiao Yuan Jing Zhang Yuan Zou Zhijuan Mao Yongjie Xiong Zhe Min Manli Yan Cong-Yi Wang Zheng Xue Source Type: research

The small molecule inhibitor PR-619 protects retinal ganglion cells against glutamate excitotoxicity
This study was designed to investigate the role of PR-619 in regulating mitophagy of RGCs under glutamate excitotoxicity. Primary cultured RGCs were incubated with PR-619 or vehicle control in the excitotoxicity model of 100 µM glutamate treatment. Mitochondrial membrane potential was assessed by JC-1 assay. Cytotoxicity of RGCs was measured by LDH activity. Proteins levels of parkin, optineurin, LAMP1, Bax, Bcl-2 and the LC3-II/I ratio were analyzed by western blot. The distribution and morphology of mitochondria in RGCs was stained by MitoTracker and antibody against mitochondria membrane protein, and examined by confoc...
Source: NeuroReport - October 6, 2020 Category: Neurology Tags: Degeneration and Repair Source Type: research

Brain Dicer1 Is Down-Regulated in a Mouse Model of Alzheimer ’s Disease Via Aβ42-Induced Repression of Nuclear Factor Erythroid 2-Related Factor 2
This study may open new avenues for investigating potential pathognomonics and pathogenesis in AD.
Source: Molecular Neurobiology - September 24, 2020 Category: Neurology Source Type: research

Protective effects of ethyl gallate on H 2 O 2 -induced mitochondrial dysfunction in PC12 cells
AbstractOxidative stress has been suggested to play an important role in neuronal injury. Ethyl gallate (EG) is the ethyl ester of gallic acid which has been acknowledged as an antioxidant. We previously demonstrated that EG effectively inhibited H2O2-induced cytotoxicity and decreased the ROS levels in PC12 cells, while the relevant mechanisms of action of this compound remain largely uncharacterized. The present study was carried out in an attempt to clarify the underlying mechanisms of EG against H2O2-induced neurotoxicity in PC12 cells. EG pretreatment attenuated H2O2-induced mitochondrial dysfunction as indicated by t...
Source: Metabolic Brain Disease - February 12, 2019 Category: Neurology Source Type: research

FOXRED1 silencing in mice: a possible animal model for Leigh syndrome
AbstractLeigh syndrome (LS) is one of the most puzzling mitochondrial disorders, which is also known as subacute necrotizing encephalopathy. It has an incidence of 1 in 77,000 live births worldwide with poor prognosis. Currently, there is a poor understanding of the underlying pathophysiological mechanisms of the disease without any available effective treatment. Hence, the inevitability for developing suitable animal and cellular models needed for the development of successful new therapeutic modalities. In this short report, we blocked FOXRED1 gene with small interfering RNA (siRNA) using C57bl/6 mice. Results showed neu...
Source: Metabolic Brain Disease - November 3, 2018 Category: Neurology Source Type: research

Inhibition of the Nrf2/HO-1 Axis Suppresses the Mitochondria-Related Protection Promoted by Gastrodin in Human Neuroblastoma Cells Exposed to Paraquat
AbstractMitochondria are double-membrane organelles involved in the transduction of energy from different metabolic substrates into adenosine triphosphate (ATP) in mammalian cells. The oxidative phosphorylation system is comprised by the activity of the respiratory chain and the complex V (ATP synthase/ATPase). This system is dependent on oxygen gas (O2) in order to maintain a flux of electrons in the respiratory chain, since O2 is the final acceptor of these electrons. Electron leakage from this complex system leads to the continuous generation of reactive species in the cells. The mammalian cells exhibit certain mechanis...
Source: Molecular Neurobiology - July 11, 2018 Category: Neurology Source Type: research

Silencing of LncRNA BDNF-AS attenuates A β25-35-induced neurotoxicity in PC12 cells by suppressing cell apoptosis and oxidative stress.
CONCLUSION: Silencing BDNF-AS exerts protective functions to increase the viability, inhibit the apoptosis and oxidative stress of Aβ25-35-induced PC12 cells by negative regulation of BDNF. ABBREVIATIONS: Aβ25-35: amyloid beta peptide 25-35; AD: Alzheimer's disease; LncRNA BDNF-AS: long non-coding RNA brain-derived neurotrophic factor anti-sense; OS: Oxidative stress. PMID: 29902125 [PubMed - as supplied by publisher]
Source: Neurological Research - June 16, 2018 Category: Neurology Tags: Neurol Res Source Type: research

The MKK7 inhibitor peptide GADD45 β-I attenuates ER stress-induced mitochondrial dysfunction in HT22 cells: involvement of JNK-Wnt pathway.
The MKK7 inhibitor peptide GADD45β-I attenuates ER stress-induced mitochondrial dysfunction in HT22 cells: involvement of JNK-Wnt pathway. Brain Res. 2018 Apr 20;: Authors: Xu GH, Song BJ, Liu D, Chen YH, Zhou Y, Liu WB, Li H, Long TL, Zhang R, Liu W Abstract JNK, a member of the mitogen activated protein kinases (MAPKs) superfamily, plays a key role in cell death in many neurological disorders, but systemic inhibition of JNK has detrimental side effects. JNK can be regulated by two direct upstream kinases: MAPK kinase 4 (MKK4) and MAPK kinase 7 (MKK7). Here, we investigated the effect of GADD45β-I,...
Source: Brain Research - April 20, 2018 Category: Neurology Authors: Xu GH, Song BJ, Liu D, Chen YH, Zhou Y, Liu WB, Li H, Long TL, Zhang R, Liu W Tags: Brain Res Source Type: research

Knockdown of long noncoding antisense RNA brain-derived neurotrophic factor attenuates hypoxia/reoxygenation-induced nerve cell apoptosis through the BDNF–TrkB–PI3K/Akt signaling pathway
Brain-derived neurotrophic factor (BDNF) plays an important role in neuronal cell apoptosis. The antisense RNA of brain-derived neurotrophic factor (BDNF-AS) is a natural antisense transcript that is transcribed opposite the gene that encodes BDNF. The aim of this study was to determine whether knockdown of BDNF-AS can suppress hypoxia/reoxygenation (H/R)-induced neuronal cell apoptosis and whether this is mediated by the BDNF–TrkB–PI3K/Akt pathway. We detected the expression of BDNF and BDNF-AS in brain tissue from 20 patients with cerebral infarction and five patients with other diseases (but no cerebral ischemia). W...
Source: NeuroReport - September 1, 2017 Category: Neurology Tags: Cellular, Molecular and Developmental Neuroscience Source Type: research

Mesenchymal Stem Cell-Derived Factors Restore Function to Human Frataxin-Deficient Cells
AbstractFriedreich ’s ataxia is an inherited neurological disorder characterised by mitochondrial dysfunction and increased susceptibility to oxidative stress. At present, no therapy has been shown to reduce disease progression. Strategies being trialled to treat Friedreich’s ataxia include drugs that improve mito chondrial function and reduce oxidative injury. In addition, stem cells have been investigated as a potential therapeutic approach. We have used siRNA-induced knockdown of frataxin in SH-SY5Y cells as an in vitro cellular model for Friedreich’s ataxia. Knockdown of frataxin protein expression to l evels det...
Source: The Cerebellum - April 29, 2017 Category: Neurology Source Type: research

Pinocembrin Suppresses H 2 O 2 -Induced Mitochondrial Dysfunction by a Mechanism Dependent on the Nrf2/HO-1 Axis in SH-SY5Y Cells
AbstractMitochondria are susceptible to redox impairment, which has been associated with neurodegeneration. These organelles are both a source and target of reactive species. In that context, there is increasing interest in finding natural compounds that modulate mitochondrial function and mitochondria-related signaling in order to prevent or to treat diseases involving mitochondrial impairment. Herein, we investigated whether and how pinocembrin (PB) would prevent mitochondrial dysfunction elicited by the exposure of human neuroblastoma SH-SY5Y cells to hydrogen peroxide (H2O2). PB (25  μM) was administrated for 4 h be...
Source: Molecular Neurobiology - January 12, 2017 Category: Neurology Source Type: research

Upregulation of Mitochondrial Biogenesis in Neuronal Cells via CREB-Mediated Transcription of TFAM (S20.007)
Conclusions: These results describe a novel property of RNS60 of stimulating mitochondrial biogenesis via PI 3-kinase-CREB-mediated up-regulation of TFAM, which may be of therapeutic benefit in different neurodegenerative disorders. This study has been supported by Revalesio Corporation, Tacoma, WA.Disclosure: Dr. Kundu has nothing to disclose. Dr. Chandra has nothing to disclose. Dr. Rangasamy has nothing to disclose. Dr. Ghosh has nothing to disclose. Dr. Watson has received personal compensation for activities with Revalesio Corporation as an employee. Dr. Pahan has nothing to disclose.
Source: Neurology - April 3, 2016 Category: Neurology Authors: Kundu, M., Chandra, G., Rangasamy, S., Ghosh, S., Watson, R., Pahan, K. Tags: General Neurology Source Type: research

UDCA exerts beneficial effect on mitochondrial dysfunction in LRRK2G2019S carriers and in vivo
Conclusion: There is clear preclinical impairment of mitochondrial function in NM-LRRK2G2019S that is distinct from the mitochondrial impairment observed in parkin-related PD. The beneficial effect of UDCA on mitochondrial function in both NM-LRRK2G2019S and M-LRRK2G2019S as well as on the function of dopaminergic neurons expressing LRRK2G2019S suggests that UDCA is a promising drug for future neuroprotective trials.
Source: Neurology - September 7, 2015 Category: Neurology Authors: Mortiboys, H., Furmston, R., Bronstad, G., Aasly, J., Elliott, C., Bandmann, O. Tags: All Movement Disorders, Parkinson's disease/Parkinsonism ARTICLE Source Type: research

Elevation of Sestrin-2 expression attenuates Sevoflurane induced neurotoxicity
In this study, our results indicated that administration of Sevoflurane elevated the gene and protein expression of Sestrin-2 in a dose dependent manner in human neuroblastoma M17 cells. It was shown that silence of Sestrin-2 by small RNA interference (siRNA) ominously exacerbated the increase in intracellular ROS and reduction of SOD activity induced by Sevoflurane treatment. Notably, knockdown of Sestrin-2 in M17 cells significantly increases the number of apoptotic cells after treatment with Sevoflurane. Mechanistically, we also found that Sevoflurane treatment resulted in a reduced amount of the cytosolic anti-apoptoti...
Source: Metabolic Brain Disease - September 5, 2015 Category: Neurology Source Type: research