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Mitochondrial dysfunction neurodegenerative disease

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https://www.readbyqxmd.com/read/28918091/opposed-effects-of-dityrosine-formation-in-soluble-and-aggregated-%C3%AE-synuclein-on-fibril-growth
#1
Michael M Wördehoff, Hamed Shaykhalishahi, Luca Groß, Lothar Gremer, Matthias Stoldt, Alexander K Buell, Dieter Willbold, Wolfgang Hoyer
Parkinson's disease (PD) is the second most common neurodegenerative disease. It is characterized by aggregation of the protein α-synuclein (α-syn) in Lewy bodies, mitochondrial dysfunction, and increased oxidative stress in the substantia nigra. Oxidative stress leads to several modifications of biomolecules including dityrosine (DiY)-crosslinking in proteins, which has recently been detected in α-syn in Lewy bodies from PD patients. Here we report that α-syn is highly susceptible to UV-induced DiY formation...
September 13, 2017: Journal of Molecular Biology
https://www.readbyqxmd.com/read/28917260/roles-of-sigma-1-receptors-on-mitochondrial-functions-relevant-to-neurodegenerative-diseases
#2
REVIEW
Tzu-Yu Weng, Shang-Yi Anne Tsai, Tsung-Ping Su
The sigma-1 receptor (Sig-1R) is a chaperone that resides mainly at the mitochondrion-associated endoplasmic reticulum (ER) membrane (called the MAMs) and acts as a dynamic pluripotent modulator in living systems. At the MAM, the Sig-1R is known to play a role in regulating the Ca(2+) signaling between ER and mitochondria and in maintaining the structural integrity of the MAM. The MAM serves as bridges between ER and mitochondria regulating multiple functions such as Ca(2+) transfer, energy exchange, lipid synthesis and transports, and protein folding that are pivotal to cell survival and defense...
September 16, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28914791/quercetin-attenuates-manganese-induced-neuroinflammation-by-alleviating-oxidative-stress-through-regulation-of-apoptosis-inos-nf-%C3%AE%C2%BAb-and-ho-1-nrf2-pathways
#3
Entaz Bahar, Ji-Ye Kim, Hyonok Yoon
Manganese (Mn) is an essential trace element required for the development of human body and acts as an enzyme co-factor or activator for various reactions of metabolism. While essential in trace amounts, excessive Mn exposure can result in toxic accumulations in human brain tissue and resulting extrapyramidal symptoms called manganism similar to idiopathic Parkinson's disease (PD). Quercetin (QCT) has been demonstrated to play an important role in altering the progression of neurodegenerative diseases by protecting against oxidative stress...
September 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28903070/role-of-the-ampk-pathway-in-promoting-autophagic-flux-via-modulating-mitochondrial-dynamics-in-neurodegenerative-diseases-insight-into-prion-diseases
#4
REVIEW
Syed Zahid Ali Shah, Deming Zhao, Tariq Hussain, Lifeng Yang
Neurons are highly energy demanding cells dependent on the mitochondrial oxidative phosphorylation system. Mitochondria generate energy via respiratory complexes that constitute the electron transport chain. Adenosine triphosphate depletion or glucose starvation act as a trigger for the activation of adenosine monophosphate-activated protein kinase (AMPK). AMPK is an evolutionarily conserved protein that plays an important role in cell survival and organismal longevity through modulation of energy homeostasis and autophagy...
September 10, 2017: Ageing Research Reviews
https://www.readbyqxmd.com/read/28883016/mitochondrial-dynamics-in-parkinson-s-disease-a-role-for-%C3%AE-synuclein
#5
REVIEW
Victorio M Pozo Devoto, Tomas L Falzone
The distinctive pathological hallmarks of Parkinson's disease are the progressive death of dopaminergic neurons and the intracellular accumulation of Lewy bodies enriched in α-synuclein protein. Several lines of evidence from the study of sporadic, familial and pharmacologically induced forms of human Parkinson's disease also suggest that mitochondrial dysfunction plays an important role in disease progression. Although many functions have been proposed for α-synuclein, emerging data from human and animal models of Parkinson's disease highlight a role for α-synuclein in the control of neuronal mitochondrial dynamics...
September 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28882140/role-of-mitochondrial-dysfunction-and-dysregulation-of-ca-2-homeostasis-in-the-pathophysiology-of-insulin-resistance-and-type-2-diabetes
#6
REVIEW
Chih-Hao Wang, Yau-Huei Wei
Metabolic diseases such as obesity, type 2 diabetes (T2D) and insulin resistance have attracted great attention from biomedical researchers and clinicians because of the astonishing increase in its prevalence. Decrease in the capacity of oxidative metabolism and mitochondrial dysfunction are a major contributor to the development of these metabolic disorders. Recent studies indicate that alteration of intracellular Ca(2+) levels and downstream Ca(2+)-dependent signaling pathways appear to modulate gene transcription and the activities of many enzymes involved in cellular metabolism...
September 7, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28880525/mitoneet-cisd1-knockout-mice-show-signs-of-striatal-mitochondrial-dysfunction-and-a-parkinson-s-disease-phenotype
#7
Werner J Geldenhuys, Stanley A Benkovic, Li Lin, Heather M Yonutas, Samuel D Crish, Patrick G Sullivan, Altaf S Darvesh, Candice M Brown, Jason Richardson
Mitochondrial dysfunction is thought to play a significant role in neurodegeneration observed in Parkinson's disease (PD), yet the mechanisms underlying this pathology remain unclear. Here, we demonstrate that loss of mitoNEET (CISD1), an iron-sulfur containing protein which regulates mitochondrial bioenergetics, results in mitochondrial dysfunction and loss of striatal dopamine and tyrosine hydroxylase. Mitochondria isolated from mice lacking mitoNEET were dysfunctional as revealed by elevated reactive oxygen species (ROS) and reduced capacity to produce ATP...
September 7, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/28877188/mitochondrial-superclusters-influence-age-of-onset-of-parkinson-s-disease-in-a-gender-specific-manner-in-the-cypriot-population-a-case-control-study
#8
Andrea Georgiou, Christiana A Demetriou, Alexandros Heraclides, Yiolanda P Christou, Eleni Leonidou, Panayiotis Loukaides, Elena Yiasoumi, Dimitris Panagiotou, Panayiotis Manoli, Pippa Thomson, Maria A Loizidou, Andreas Hadjisavvas, Eleni Zamba-Papanicolaou
BACKGROUND: Despite evidence supporting an involvement of mitochondrial dysfunction in the pathogenesis of some neurodegenerative disorders, there are inconsistent findings concerning mitochondrial haplogroups and their association to neurodegenerative disorders, including idiopathic Parkinson's disease (PD). METHODS: To test this hypothesis for the Greek-Cypriot population, a cohort of 230 PD patients and 457 healthy matched controls were recruited. Mitochondrial haplogroup distributions for cases and controls were determined...
2017: PloS One
https://www.readbyqxmd.com/read/28867595/mitochondrial-ferredoxin-determines-vulnerability-of-cells-to-copper-excess
#9
Cindy Vallières, Sara L Holland, Simon V Avery
The essential micronutrient copper is tightly regulated in organisms, as environmental exposure or homeostasis defects can cause toxicity and neurodegenerative disease. The principal target(s) of copper toxicity have not been pinpointed, but one key effect is impaired supply of iron-sulfur (FeS) clusters to the essential protein Rli1 (ABCE1). Here, to find upstream FeS biosynthesis/delivery protein(s) responsible for this, we compared copper sensitivity of yeast-overexpressing candidate targets. Overexpression of the mitochondrial ferredoxin Yah1 produced copper hyper-resistance...
August 24, 2017: Cell Chemical Biology
https://www.readbyqxmd.com/read/28865316/oxidative-species-induced-excitonic-transport-in-tubulin-aromatic-networks-potential-implications-for-neurodegenerative-disease
#10
P Kurian, T O Obisesan, T J A Craddock
Oxidative stress is a pathological hallmark of neurodegenerative tauopathic disorders such as Alzheimer's disease and Parkinson's disease-related dementia, which are characterized by altered forms of the microtubule-associated protein (MAP) tau. MAP tau is a key protein in stabilizing the microtubule architecture that regulates neuron morphology and synaptic strength. When MAP tau is degraded in tauopathic disorders, neuron dysfunction results. The precise role of reactive oxygen species (ROS) in the tauopathic disease process, however, is poorly understood...
August 24, 2017: Journal of Photochemistry and Photobiology. B, Biology
https://www.readbyqxmd.com/read/28863942/mitochondrial-dysfunction-in-the-app-psen1-mouse-model-of-alzheimer-s-disease-and-a-novel-protective-role-for-ascorbate
#11
Shilpy Dixit, Joshua P Fessel, Fiona E Harrison
Mitochondrial dysfunction is elevated in very early stages of Alzheimer's disease and exacerbates oxidative stress, which contributes to disease pathology. Mitochondria were isolated from 4-month-old wild-type mice, transgenic mice carrying the APPSWE and PSEN1dE9 mutations, mice with decreased brain and mitochondrial ascorbate (vitamin C) via heterozygous knockout of the sodium dependent vitamin C transporter (SVCT2(+/-)) and transgenic APP/PSEN1 mice with heterozygous SVCT2 expression. Mitochondrial isolates from SVCT2(+/-) mice were observed to consume less oxygen using high-resolution respirometry, and also exhibited decreased mitochondrial membrane potential compared to wild type isolates...
August 31, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28860846/ambroxol-for-the-treatment-of-fibromyalgia-science-or-fiction
#12
Kai-Uwe Kern, Myriam Schwickert
Fibromyalgia appears to present in subgroups with regard to biological pain induction, with primarily inflammatory, neuropathic/neurodegenerative, sympathetic, oxidative, nitrosative, or muscular factors and/or central sensitization. Recent research has also discussed glial activation or interrupted dopaminergic neurotransmission, as well as increased skin mast cells and mitochondrial dysfunction. Therapy is difficult, and the treatment options used so far mostly just have the potential to address only one of these aspects...
2017: Journal of Pain Research
https://www.readbyqxmd.com/read/28860335/activation-mechanisms-of-the-e3-ubiquitin-ligase-parkin
#13
REVIEW
Nikhil Panicker, Valina L Dawson, Ted M Dawson
Monogenetic, familial forms of Parkinson's disease (PD) only account for 5-10% of the total number of PD cases, but analysis of the genes involved therein is invaluable to understanding PD-associated neurodegenerative signaling. One such gene, parkin, encodes a 465 amino acid E3 ubiquitin ligase. Of late, there has been considerable interest in the role of parkin signaling in PD and in identifying its putative substrates, as well as the elucidation of the mechanisms through which parkin itself is activated...
August 30, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28852382/mitochondrial-quality-control-in-amyotrophic-lateral-sclerosis-towards-a-common-pathway
#14
REVIEW
Bilal Khalil, Jean-Charles Liévens
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder characterized by loss of upper and lower motor neurons. Different mechanisms contribute to the disease initiation and progression, including mitochondrial dysfunction which has been proposed to be a central determinant in ALS pathogenesis. Indeed, while mitochondrial defects have been mainly described in ALS-linked SOD1 mutants, it is now well established that mitochondria become also dysfunctional in other ALS conditions. In such context, the mitochondrial quality control system allows to restore normal functioning of mitochondria and to prevent cell death, by both eliminating and replacing damaged mitochondrial components or by degrading the entire organelle through mitophagy...
July 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28844784/dieldrin-induced-neurotoxicity-involves-impaired-mitochondrial-bioenergetics-and-an-endoplasmic-reticulum-stress-response-in-rat-dopaminergic-cells
#15
Jordan T Schmidt, Anna Rushin, Jonna Boyda, Christopher Laurence Souders, Christopher J Martyniuk
Mitochondria are sensitive targets of environmental chemicals. Dieldrin (DLD) is an organochlorine pesticide that remains a human health concern due to high lipid bioaccumulation, and it has been epidemiologically associated to an increased risk for Parkinson's disease (PD). As mitochondrial dysfunction is involved in the etiology of PD, this study aimed to determine whether DLD impaired mitochondrial bioenergetics in dopaminergic cells. Rat immortalized dopaminergic N27 cells were treated for 24 or 48h with one dose of either a solvent control, 2...
August 24, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28840556/mitochondrial-diseases-as-model-of-neurodegeneration
#16
Laila A Selim, Heba Hassaan
"Mitochondria" partially autonomous sophisticated cellular organelle involved in a wide range of crucial cellular functions, well known as the power house of the cell where ATP (adenosine triphosphate) production takes place, that is the cellular source of energy.Mitochondria has its own genome, however proper functioning of the mitochondria is dependent upon the coordinated expression of both nuclear and mitochondrial encoded gene products. Peculiar maternal inheritance of mitochondrial DNA has led the scientists to think about mitochondrial donation as a solution to maternally inherited mitochondriopathy "Three parent baby", raising many ethical and scientific issues, concerns about safety of the procedure, long term outcome and effect of genetic modification are still questionable...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28840449/from-mitochondrial-function-to-neuroprotection-an-emerging-role-for-methylene-blue
#17
REVIEW
Donovan Tucker, Yujiao Lu, Quanguang Zhang
Methylene blue (MB) is a well-established drug with a long history of use, owing to its diverse range of use and its minimal side effect profile. MB has been used classically for the treatment of malaria, methemoglobinemia, and carbon monoxide poisoning, as well as a histological dye. Its role in the mitochondria, however, has elicited much of its renewed interest in recent years. MB can reroute electrons in the mitochondrial electron transfer chain directly from NADH to cytochrome c, increasing the activity of complex IV and effectively promoting mitochondrial activity while mitigating oxidative stress...
August 24, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28838841/mitochondria-as-pharmacological-targets-in-down-syndrome
#18
REVIEW
Daniela Valenti, Nady Braidy, Domenico De Rasmo, Anna Signorile, Leonardo Rossi, Atanas Georgiev Atanasov, Mariateresa Volpicella, Alexandra Henrion-Caude, Seyed Mohammad Nabavi, Rosa Anna Vacca
Mitochondria play a pivotal role in cellular energy-generating processes and are considered master regulators of cell life and death fate. Mitochondrial function integrates signalling networks in several metabolic pathways controlling neurogenesis and neuroplasticity. Indeed, dysfunctional mitochondria and mitochondrial-dependent activation of intracellular stress cascades are critical initiating events in many human neurodegenerative or neurodevelopmental diseases including Down syndrome (DS). It is well established that trisomy of human chromosome 21 can cause DS...
August 31, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28837882/a-reciprocal-relationship-between-reactive-oxygen-species-and-mitochondrial-dynamics-in-neurodegeneration
#19
Clara Hiu-Ling Hung, Sally Shuk-Yee Cheng, Yuen-Ting Cheung, Suthicha Wuwongse, Natalie Qishan Zhang, Yuen-Shan Ho, Simon Ming-Yuen Lee, Raymond Chuen-Chung Chang
Mitochondrial fragmentation due to fission/fusion imbalance has often been linked to mitochondrial dysfunction and apoptosis in neurodegeneration. Conventionally, it is believed that once mitochondrial morphology shifts away from its physiological tubular form, mitochondria become defective and downstream apoptotic signaling pathways are triggered. However, our study shows that beta-amyloid (Aβ) induces morphological changes in mitochondria where they become granular-shaped and are distinct from fragmented mitochondria in terms of both morphology and functions...
August 12, 2017: Redox Biology
https://www.readbyqxmd.com/read/28837411/regulation-of-autophagy-mitochondrial-dynamics-and-cellular-bioenergetics-by-4-hydroxynonenal-in-primary-neurons
#20
Matthew Dodson, Willayat Wani, Matthew Redmann, Gloria A Benavides, Michelle S Johnson, Xiaosen Ouyang, Stacey S Cofield, Kasturi Mitra, Victor Darley-Usmar, Jianhua Zhang
The production of reactive species contributes to the age-dependent accumulation of dysfunctional mitochondria and protein aggregates, all of which are associated with neurodegeneration. A putative mediator of these effects is the lipid peroxidation product 4-hydroxynonenal (4-HNE), which has been shown to inhibit mitochondrial function, and accumulate in the postmortem brains of patients suffering from neurodegenerative diseases. This deterioration in mitochondrial quality could be due to direct effects on mitochondrial proteins, or through perturbation of the macroautophagy/autophagy pathway, which plays an essential role in removing damaged mitochondria...
August 24, 2017: Autophagy
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