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Mitochondrial dysfunction neurodegenerative disease

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https://www.readbyqxmd.com/read/28443065/hemoglobin-improved-protection-in-cultured-cerebral-cortical-astroglial-cells-inhibition-of-oxidative-stress-and-caspase-activation
#1
Fatma Amri, Ikram Ghouili, Marie-Christine Tonon, Mohamed Amri, Olfa Masmoudi-Kouki
Oxidative stress plays a major role in triggering astroglial cell death in diverse neuropathological conditions such as ischemia and neurodegenerative diseases. Numerous studies indicate that hemoglobin (Hb) is expressed in both resting and reactive glia cells, but nothing is known regarding a possible role of Hb on astroglial cell survival. Thus, the purpose of the present study was to investigate the potential glioprotective effect of Hb on hydrogen peroxide (H2O2)-induced oxidative stress and apoptosis in cultured rat astrocytes...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28438530/ferulic-acid-attenuates-brain-microvascular-endothelial-cells-damage-caused-by-oxygen-glucose-deprivation-via-punctate-mitochondria-dependent-mitophagy
#2
Jun-Li Chen, Wen-Jun Duan, Si Luo, Shi Li, Xiao-Hui Ma, Bo-Nan Hou, Shu-Yi Cheng, Shu-Huan Fang, Qi Wang, Shui-Qing Huang, Yun-Bo Chen
Ferulic acid (FA) has an important effect on scavenging free radicals, which is related to the alleviation of various neurodegenerative diseases. However, there are few studies about its effects on vascular dementia. In this study, we demonstrated the effect of FA on oxidative damage of brain microvascular endothelial cells (BMECs) which underwent oxygen-glucose deprivation (OGD) for 2 hours. Our data showed that FA significantly reversed the oxidative stress state of OGD-treated BMECs and reduced mitochondrial dysfunction...
April 21, 2017: Brain Research
https://www.readbyqxmd.com/read/28436392/the-dna-damage-response-in-neurons-die-by-apoptosis-or-survive-in-a-senescence-like-state
#3
Edward Fielder, Thomas von Zglinicki, Diana Jurk
Neurons are exposed to high levels of DNA damage from both physiological and pathological sources. Neurons are post-mitotic and their loss cannot be easily recovered from; to cope with DNA damage a complex pathway called the DNA damage response (DDR) has evolved. This recognizes the damage, and through kinases such as ataxia-telangiectasia mutated (ATM) recruits and activates downstream factors that mediate either apoptosis or survival. This choice between these opposing outcomes integrates many inputs primarily through a number of key cross-road proteins, including ATM, p53, and p21...
April 18, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28429146/clinical-validity-of-biochemical-and-molecular-analysis-in-diagnosing-leigh-syndrome-a-study-of-106-japanese-patients
#4
Erika Ogawa, Masaru Shimura, Takuya Fushimi, Makiko Tajika, Keiko Ichimoto, Ayako Matsunaga, Tomoko Tsuruoka, Mika Ishige, Tatsuo Fuchigami, Taro Yamazaki, Masato Mori, Masakazu Kohda, Yoshihito Kishita, Yasushi Okazaki, Shori Takahashi, Akira Ohtake, Kei Murayama
Leigh syndrome (LS) is a progressive neurodegenerative disorder of infancy and early childhood. It is clinically diagnosed by typical manifestations and characteristic computed tomography (CT) or magnetic resonance imaging (MRI) studies. Unravelling mitochondrial respiratory chain (MRC) dysfunction behind LS is essential for deeper understanding of the disease, which may lead to the development of new therapies and cure. The aim of this study was to evaluate the clinical validity of various diagnostic tools in confirming MRC disorder in LS and Leigh-like syndrome (LL)...
April 20, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28420982/tau-oligomers-cytotoxicity-propagation-and-mitochondrial-damage
#5
REVIEW
Scott S Shafiei, Marcos J Guerrero-Muñoz, Diana L Castillo-Carranza
Aging has long been considered as the main risk factor for several neurodegenerative disorders including a large group of diseases known as tauopathies. Even though neurofibrillary tangles (NFTs) have been examined as the main histopathological hallmark, they do not seem to play a role as the toxic entities leading to disease. Recent studies suggest that an intermediate form of tau, prior to NFT formation, the tau oligomer, is the true toxic species. However, the mechanisms by which tau oligomers trigger neurodegeneration remain unknown...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28420370/neuroprotective-effect-of-demethoxycurcumin-a-natural-derivative-of-curcumin-on-rotenone-induced-neurotoxicity-in-sh-sy-5y-neuroblastoma-cells
#6
Muthu Ramkumar, Srinivasagam Rajasankar, Veerappan Venkatesh Gobi, Chinnasamy Dhanalakshmi, Thamilarasan Manivasagam, Arokiasamy Justin Thenmozhi, Musthafa Mohamed Essa, Ameer Kalandar, Ranganathan Chidambaram
BACKGROUND: Mitochondrial dysfunction and oxidative stress are the main toxic events leading to dopaminergic neuronal death in Parkinson's disease (PD) and identified as vital objective for therapeutic intercession. This study investigated the neuro-protective effects of the demethoxycurcumin (DMC), a derivative of curcumin against rotenone induced neurotoxicity. METHODS: SH-SY5Y neuroblastoma cells are divided into four experimental groups: untreated cells, cells incubated with rotenone (100 nM), cells treated with DMC (50 nM) + rotenone (100 nM) and DMC alone treated...
April 18, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28413833/beneficial-effects-of-a-pyrroloquinolinequinone-containing-dietary-formulation-on-motor-deficiency-cognitive-decline-and-mitochondrial-dysfunction-in-a-mouse-model-of-alzheimer-s-disease
#7
Darrell Sawmiller, Song Li, Takashi Mori, Ahsan Habib, David Rongo, Vedad Delic, Patrick C Bradshaw, R Douglas Shytle, Cyndy Sanberg, Paula Bickford, Jun Tan
Alzheimer's disease (AD), a progressive neurodegenerative disorder, is linked to oxidative stress, altered amyloid precursor protein (APP) proteolysis, tau hyperphosphorylation and the accumulation of amyloid-β (Aβ) plaques and neurofibrillary tangles (NFT). A growing body of evidence suggests that mitochondrial dysfunction can be a key promoter of all of these pathologies and predicts that restoration of mitochondrial function might be a potential therapeutic strategy for AD. Therefore, in the present study, we tested the beneficial effect of a nutraceutical formulation Nutrastem II (Nutra II), containing NT020 (a mitochondrial restorative and antioxidant proprietary formulation) and pyrroloquinolinequinone (PQQ, a stimulator of mitochondria biogenesis) in 5XFAD transgenic mice...
April 2017: Heliyon
https://www.readbyqxmd.com/read/28408132/comparison-of-the-effects-of-major-fatty-acids-present-in-the-mediterranean-diet-oleic-acid-docosahexaenoic-acid-and-in-hydrogenated-oils-elaidic-acid-on-7-ketocholesterol-induced-oxiapoptophagy-in-microglial-bv-2-cells
#8
Meryam Debbabi, Amira Zarrouk, Maryem Bezine, Wiem Meddeb, Thomas Nury, Asmaa Badreddine, El Mostafa Karym, Randa Sghaier, Lionel Bretillon, Stéphane Guyot, Mohammad Samadi, Mustapha Cherkaoui-Malki, Boubker Nasser, Mondher Mejri, Sofien Ben-Hammou, Mohamed Hammami, Gérard Lizard
Increased levels of 7-ketocholesterol (7KC), which results mainly from cholesterol auto-oxidation, are often found in the plasma and/or cerebrospinal fluid of patients with neurodegenerative diseases and might contribute to activation of microglial cells involved in neurodegeneration. As major cellular dysfunctions are induced by 7KC, it is important to identify molecules able to impair its side effects. Since consumption of olive and argan oils, and fish is important in the Mediterranean diet, the aim of the study was to determine the ability of oleic acid (OA), a major compound of olive and argan oil, and docosahexaenoic acid (DHA) present in fatty fishes, such as sardines, to attenuate 7KC-induced cytotoxic effects...
April 10, 2017: Chemistry and Physics of Lipids
https://www.readbyqxmd.com/read/28405779/multidirectional-inhibition-of-cortico-hippocampal-neurodegeneration-by-kolaviron-treatment-in-rats
#9
Olayemi Joseph Olajide, Nnaemeka Tobechukwu Asogwa, Blessing Oluwapelumi Moses, Christiana Bidemi Oyegbola
Earliest signs of neurodegenerative cascades in the course of Alzheimer's disease (AD) are seen within the prefrontal cortex (PFC) and hippocampus, with pathological evidences in both cortical structures correlating with manifestation of behavioural and cognitive deficits. Despite the enormous problems associated with AD's clinical manifestations in sufferers, therapeutic advances for the disorder are still very limited. Therefore, this study examined cortico-hippocampal microstructures in models of AD, and evaluated the possible beneficial roles of kolaviron (Kv)-a biflavonoid complex in rats...
April 13, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28403913/brilliant-blue-g-but-not-fenofibrate-treatment-reverts-hemiparkinsonian-behavior-and-restores-dopamine-levels-in-an-animal-model-of-parkinson-s-disease
#10
Enéas G Ferrazoli, Héllio D N de Souza, Isis C Nascimento, Ágatha Oliveira-Giacomelli, Telma T Schwindt, Luiz R Britto, Henning Ulrich
Parkinson's disease (PD) is a neurodegenerative disorder, characterized by the loss of dopaminergic neurons in the substantia nigra and their projections to the striatum. Several processes have been described as potential inducers of the dopaminergic neuron death, such as inflammation, oxidative stress, and mitochondrial dysfunction. However, the death of dopaminergic neurons seems to be multifactorial, and its cause remains unclear. ATP-activating purinergic receptors influence various physiological functions in the CNS, including neurotransmission...
April 13, 2017: Cell Transplantation
https://www.readbyqxmd.com/read/28400824/melatonin-and-human-mitochondrial-diseases
#11
REVIEW
Reza Sharafati-Chaleshtori, Hedayatollah Shirzad, Mahmoud Rafieian-Kopaei, Amin Soltani
Mitochondrial dysfunction is one of the main causative factors in a wide variety of complications such as neurodegenerative disorders, ischemia/reperfusion, aging process, and septic shock. Decrease in respiratory complex activity, increase in free radical production, increase in mitochondrial synthase activity, increase in nitric oxide production, and impair in electron transport system and/or mitochondrial permeability are considered as the main factors responsible for mitochondrial dysfunction. Melatonin, the pineal gland hormone, is selectively taken up by mitochondria and acts as a powerful antioxidant, regulating the mitochondrial bioenergetic function...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28400813/adenyl-cyclase-activator-forskolin-protects-against-huntington-s-disease-like-neurodegenerative-disorders
#12
Sidharth Mehan, Shaba Parveen, Sanjeev Kalra
Long term suppression of succinate dehydrogenase by selective inhibitor 3-nitropropionic acid has been used in rodents to model Huntington's disease where mitochondrial dysfunction and oxidative damages are primary pathological hallmarks for neuronal damage. Improvements in learning and memory abilities, recovery of energy levels, and reduction of excitotoxicity damage can be achieved through activation of Adenyl cyclase enzyme by a specific phytochemical forskolin. In this study, intraperitoneal administration of 10 mg/kg 3-nitropropionic acid for 15 days in rats notably reduced body weight, worsened motor cocordination (grip strength, beam crossing task, locomotor activity), resulted in learning and memory deficits, greatly increased acetylcholinesterase, lactate dehydrogenase, nitrite, and malondialdehyde levels, obviously decreased adenosine triphosphate, succinate dehydrogenase, superoxide dismutase, catalase, and reduced glutathione levels in the striatum, cortex and hippocampus...
February 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28400790/current-view-and-perspectives-in-amyotrophic-lateral-sclerosis
#13
REVIEW
Stéphane Mathis, Philippe Couratier, Adrien Julian, Philippe Corcia, Gwendal Le Masson
Amyotrophic lateral sclerosis (ALS), identified as a distinct clinical entity by Charcot since the end of the nineteenth century, is a devastating and fatal neurodegenerative disorder that affects motor neurons in the brain, brainstem and spinal cord. Survival of patients with ALS is associated with several factors such as clinical phenotype, age at onset, gender, early presence of respiratory failure, weight loss and treatment with Riluzole (the only disease-modifying drug approved for this disease). Nowadays, there is still no curative treatment for ALS: palliative care and symptomatic treatment are therefore essential components in the management of these patients...
February 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28400134/alpha-synuclein-pathology-mitochondrial-dysfunction-and-neuroinflammation-in-parkinson-s-disease
#14
REVIEW
Emily M Rocha, Briana De Miranda, Laurie H Sanders
Parkinson's disease (PD) is a complex, chronic and progressive neurodegenerative disease. While the etiology of PD is likely multifactorial, the protein α-synuclein is a central component to the pathogenesis of the disease. However, the mechanism by which α-synuclein causes toxicity and contributes to neuronal death remains unclear. Mitochondrial dysfunction is also widely considered to play a major role in the underlying mechanisms contributing to neurodegeneration in PD. This review discusses evidence for the neuropathological role for α-synuclein in the dysfunction of dopamine neurons in PD...
April 8, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28397282/brain-aging-and-neurodegeneration-from-a-mitochondrial-point-of-view
#15
REVIEW
Amandine Grimm, Anne Eckert
Aging was defined as a progressive time-related accumulation of changes responsible for or at least involved in the increased susceptibility to disease and death. The brain seems to be particularly sensitive to the aging process since the appearance of neurodegenerative diseases, including Alzheimer's disease, is exponential with the increasing age. Mitochondria were placed at the center of the "free-radical theory of aging", because these paramount organelles are not only the main producers of energy in the cells, but also to main source of reactive oxygen species (ROS)...
April 11, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28392448/effect-of-nitric-oxide-to-axonal-degeneration-in-multiple-sclerosis-via-downregulating-monocarboxylate-transporter-1-in-oligodendrocytes
#16
REVIEW
Xiaoyi Tang, Minghong Lan, Mao Zhang, Zhongxiang Yao
Multiple sclerosis (MS) is a neurodegenerative disease of the central nervous system (CNS). Axonal degeneration, one of the main pathological characteristics of MS, is affected by nitric oxide (NO). In turn, NO induces mitochondrial dysfunction of neurons and glial cells. Inadequate glucose causes monocarboxylate transporter 1 (MCT1) to transfer lactate from oligodendrocytes (OLs) to neurons, which decreases MCT1 and results in energy substrate deficit (mainly lactate) in axons. The condition gradually leads to axonal degeneration...
April 6, 2017: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/28389271/from-dysfunctional-endoplasmic-reticulum-mitochondria-coupling-to-neurodegeneration
#17
Zoi Erpapazoglou, François Mouton-Liger, Olga Corti
Over the last years, contact sites between the endoplasmic reticulum (ER) and mitochondria have attracted great attention in the study of cell homeostasis and dysfunction, especially in the context of neurodegenerative disorders. This is largely due to the critical involvement of this subcellular compartment in a plethora of vital cellular functions: Ca(2+) homeostasis, mitochondrial dynamics, transport, bioenergetics and turnover, ER stress, apoptotic signaling and inflammation. An increasing number of disease-associated proteins have been reported to physically associate with the ER-mitochondria interface, and cause structural and/or functional perturbations of this compartment...
April 5, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28386764/roles-of-tau-protein-in-health-and-disease
#18
REVIEW
Tong Guo, Wendy Noble, Diane P Hanger
Tau is well established as a microtubule-associated protein in neurons. However, under pathological conditions, aberrant assembly of tau into insoluble aggregates is accompanied by synaptic dysfunction and neural cell death in a range of neurodegenerative disorders, collectively referred to as tauopathies. Recent advances in our understanding of the multiple functions and different locations of tau inside and outside neurons have revealed novel insights into its importance in a diverse range of molecular pathways including cell signalling, synaptic plasticity, and regulation of genomic stability...
May 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28379216/nicorandil-attenuates-neuronal-mitochondrial-dysfunction-and-oxidative-stress-associated-with-murine-model-of-vascular-calcification
#19
Sriram Ravindran, Krithika Swaminathan, Abhinaya Ramesh, Gino A Kurian
Evidences suggest that the presence of chronic kidney disease (CKD) is associated with cerebrovascular diseases related cognitive decline in dialysis patients. As mitochondrial dysfunction is implicated in neurodegenerative disorders, we hypothesized that changes in brain mitochondria occur due to vascular calcification induced by renal failure and the opening of the mitochondrial potassium channel using nicorandil may prevent its dysfunction. Brain tissues from rats with vascular calcification were studied...
2017: Acta Neurobiologiae Experimentalis
https://www.readbyqxmd.com/read/28379197/abnormalities-of-mitochondrial-dynamics-in-neurodegenerative-diseases
#20
REVIEW
Ju Gao, Luwen Wang, Jingyi Liu, Fei Xie, Bo Su, Xinglong Wang
Neurodegenerative diseases are incurable and devastating neurological disorders characterized by the progressive loss of the structure and function of neurons in the central nervous system or peripheral nervous system. Mitochondria, organelles found in most eukaryotic cells, are essential for neuronal survival and are involved in a number of neuronal functions. Mitochondrial dysfunction has long been demonstrated as a common prominent early pathological feature of a variety of common neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington's disease (HD)...
April 5, 2017: Antioxidants (Basel, Switzerland)
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