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https://www.readbyqxmd.com/read/29458148/untimely-oxidative-stress-in-%C3%AE-cells-leads-to-diabetes-role-of-circadian-clock-in-%C3%AE-cell-function
#1
REVIEW
J Lee, K Ma, M Moulik, V Yechoor
Diabetes results from a loss of β-cell function. With the number of people with diabetes reaching epidemic proportions globally, understanding mechanisms that are contributing to this increasing prevalence is critical. One such factor has been circadian disruption, with shift-work, light pollution, jet-lag, increased screen time, all acting as potential contributory factors. Though circadian disruption has been epidemiologically associated with diabetes and other metabolic disorders for many decades, it is only recently that there has been a better understanding of the underlying molecular mechanisms...
February 16, 2018: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/29458125/pluripotent-stem-cells-for-uncovering-the-role-of-mitochondria-in-human-brain-function-and-dysfunction
#2
REVIEW
Annika Zink, Josef Priller, Alessandro Prigione
Mitochondrial dysfunctions are a known pathogenetic mechanism of a number of neurological and psychiatric disorders. At the same time, mutations in genes encoding for components of the mitochondrial respiratory chain cause mitochondrial diseases, which commonly exhibit neurological symptoms. Mitochondria are therefore critical for the functionality of the human nervous system. The importance of mitochondria stems from their key roles in cellular metabolism, calcium handling, redox and protein homeostasis, and overall cellular homeostasis through their dynamic network...
February 16, 2018: Journal of Molecular Biology
https://www.readbyqxmd.com/read/29457828/dihydrodiosgenin-protects-against-experimental-acute-pancreatitis-and-associated-lung-injury-through-mitochondrial-protection-and-pi3k%C3%AE-akt-inhibition
#3
Yan Shen, Li Wen, Rui Zhang, Zeliang Wei, Na Shi, Qiuyang Xiong, Qing Xia, Zhihua Xing, Zhi Zeng, Hai Niu, Wen Huang
BACKGROUND AND PURPOSE: Acute pancreatitis (AP) is a painful and burdensome exocrine pancreatic disorder with no specific drug treatment. Diosgenyl saponin extracts from Dioscorea zingiberensis C. H. Wright were reported to protect against experimental models of AP and diosgenin or its derivatives have anti-inflammatory effects in various diseases. However, the effects of diosgenin and its spiroacetal ring opened analog dihydrodiosgenin (Dydio) have not yet been determined in AP. EXPERIMENTAL APPROACH: Various concentrations of diosgenin and Dydio were tested for sodium taurocholate hydrate (Tauro)-induced necrosis in freshly isolated murine pancreatic acinar cells...
February 19, 2018: British Journal of Pharmacology
https://www.readbyqxmd.com/read/29456585/neuroinflammation-and-neurohormesis-in-the-pathogenesis-of-alzheimer-s-disease-and-alzheimer-linked-pathologies-modulation-by-nutritional-mushrooms
#4
REVIEW
Angela Trovato Salinaro, Manuela Pennisi, Rosanna Di Paola, Maria Scuto, Rosalia Crupi, Maria Teresa Cambria, Maria Laura Ontario, Mario Tomasello, Maurizio Uva, Luigi Maiolino, Edward J Calabrese, Salvatore Cuzzocrea, Vittorio Calabrese
Human life develops and expands not only in time and space, but also in the retrograde permanent recollection and interweaving of memories. Therefore, individual human identity depends fully on a proper access to the autobiographical memory. Such access is hindered or lost under pathological conditions such as Alzheimer's disease, including recently associated oxidant pathologies, such as ocular neural degeneration occurring in glaucoma or neurosensorial degeneration occurring in Menière's disease. Oxidative stress and altered antioxidant systems have been suggested to play a role in the aetiology of major neurodegenerative disorders, and altered expression of genes sensing oxidative stress, as well as decreased cellular stress response mechanisms could synergistically contribute to the course of these oxidant disorders...
2018: Immunity & Ageing: I & A
https://www.readbyqxmd.com/read/29456504/a-review-on-central-nervous-system-effects-of-gastrodin
#5
REVIEW
Yuan Liu, Jialiang Gao, Min Peng, Hongyan Meng, Hongbo Ma, Pingping Cai, Yuan Xu, Qiong Zhao, Guomin Si
Rhizoma Gastrodiae (also known as Tian ma ), the dried rhizome of Gastrodia elata Blume, is a famous Chinese herb that has been traditionally used for the treatment of headache, dizziness, spasm, epilepsy, stoke, amnesia and other disorders for centuries. Gastrodin, a phenolic glycoside, is the main bioactive constituent of Rhizoma Gastrodiae . Since identified in 1978, gastrodin has been extensively investigated on its pharmacological properties. In this article, we reviewed the central nervous system (CNS) effects of gastrodin in preclinical models of CNS disorders including epilepsy, Alzheimer's disease, Parkinson's disease, affective disorders, cerebral ischemia/reperfusion, cognitive impairment as well as the underlying mechanisms involved and, where possible, clinical data that support the pharmacological activities...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29455419/further-aspects-of-toxoplasma-gondii-elimination-in-the-presence-of-metals
#6
Laís Pessanha de Carvalho, Edésio José Tenório de Melo
Toxoplasma gondii, the etiological agent of toxoplasmosis, infects nucleated cells and then resides and multiplies within a parasitophorous vacuole. For this purpose, the parasite secretes many virulence factors for the purpose of invading and subverting the host microbicidal defenses in order to facilitate its survival in the intracellular milieu. Essential metals are structural components of proteins and enzymes or cofactors of enzymatic reactions responsible for these parasitic survival mechanisms. However, an excess of non-essential or essential metals can lead to parasite death...
February 18, 2018: Parasitology Research
https://www.readbyqxmd.com/read/29454676/opa1-how-much-do-we-know-to-approach-therapy
#7
REVIEW
Valentina Del Dotto, Mario Fogazza, Guy Lenaers, Michela Rugolo, Valerio Carelli, Claudia Zanna
OPA1 is a GTPase that controls several functions, such as mitochondrial dynamics and energetics, mtDNA maintenance and cristae integrity. In the last years, there have been described other cellular pathways and mechanisms involving OPA1 directly or through its interaction. All this new information, by implementing our knowledge on OPA1 is instrumental to elucidating the pathogenic mechanisms of OPA1 mutations. Indeed, these are associated with dominant optic atrophy (DOA), one of the most common inherited optic neuropathies, and with an increasing number of heterogeneous neurodegenerative disorders...
February 15, 2018: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29454073/the-accumulation-of-assembly-intermediates-of-the-mitochondrial-complex-i-matrix-arm-is-reduced-by-limiting-glucose-uptake-in-a-neuronal-like-model-of-melas-syndrome
#8
Guillaume Geffroy, Rayane Benyahia, Samuel Frey, Valerie Desquiret-Dumas, Naig Gueguen, Celine Bris, Sophie Belal, Aurore Inisan, Aurelie Renaud, Arnaud Chevrollier, Daniel Henrion, Dominique Bonneau, Franck Letournel, Guy Lenaers, Pascal Reynier, Vincent Procaccio
Ketogenic diet (KD) which combined carbohydrate restriction and the addition of ketone bodies has emerged as an alternative metabolic intervention used as an anticonvulsant therapy or to treat different types of neurological or mitochondrial disorders including MELAS syndrome. MELAS syndrome is a severe mitochondrial disease mainly due to the m.3243A > G mitochondrial DNA mutation. The broad success of KD is due to multiple beneficial mechanisms with distinct effects of very low carbohydrates and ketones...
February 14, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29454001/brain-bioenergetics-in-rats-with-acute-hyperphenylalaninemia
#9
Nádia Weber Dimer, Bruna Klippel Ferreira, Jotele Fontana Agostini, Maria Luiza Gomes, Luiza Wilges Kist, Fernanda Malgarin, Milena Carvalho-Silva, Lara Mezari Gomes, Joyce Rebelo, Marisa Jádna Silva Frederico, Fátima Regina Mena Barreto Silva, Eduardo Pacheco Rico, Mauricio Reis Bogo, Emilio Luiz Streck, Gustavo Costa Ferreira, Patrícia Fernanda Schuck
Phenylketonuria (PKU) is a disorder of phenylalanine (Phe) metabolism caused by deficient phenylalanine hydroxylase activity. The deficiency results in increased levels of Phe and its metabolites in fluids and tissues of patients. PKU patients present neurological signals and symptoms including hypomyelination and intellectual deficit. This study assessed brain bioenergetics at 1 h after acute Phe administration to induce hyperphenylalaninemia (HPA) in rats. Wistar rats were randomized in two groups: HPA animals received a single subcutaneous administration of Phe (5...
February 14, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29453877/significance-of-mitochondria-on-cardiometabolic-syndromes
#10
REVIEW
Nair Anupama, G Sindhu, K G Raghu
Metabolic syndromes (MS) are a cluster of disorders like obesity, hypertension, dyslipidemia, and diabetes. Cardiometabolic syndrome (CMS), a branch of MS, is a group of diseases affecting cardiovascular, renal, metabolic, prothrombotic and inflammatory abnormalities due to defects in energy metabolism. Since the emergence of molecular biology and the discovery of pathogenic mitochondrial DNA defect in the 1980s, research advances has revealed a number of common human disease involving mitochondrial dysfunction...
February 17, 2018: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/29453449/functional-analysis-of-slc39a8-mutations-and-their-implications-for-manganese-deficiency-and-mitochondrial-disorders
#11
Eun-Kyung Choi, Trang-Tiffany Nguyen, Neil Gupta, Shigeki Iwase, Young Ah Seo
SLC39A8 encodes ZIP8, a divalent metal ion transporter. Mutations in the SLC39A8 gene are associated with congenital disorder of glycosylation type II and Leigh syndrome. Notably, affected patients with both disorders exhibited severe manganese (Mn) deficiency. The cellular function of human SLC39A8 (hSLC39A8) and the mechanisms by which mutations in this protein lead to human diseases are unclear. Herein, we show that hSLC39A8 mediates 54 Mn uptake by the cells, and its expression is regulated by Mn. While expression of wild-type hSLC39A8 increased 54 Mn uptake activity, disease-associated mutations abrogated the ability of the transporter to mediate Mn uptake into the cells, thereby providing a causal link to severe Mn deficiency...
February 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29453441/circulating-cell-free-mitochondrial-dna-but-not-leukocyte-mitochondrial-dna-copy-number-is-elevated-in-major-depressive-disorder
#12
Daniel Lindqvist, Owen M Wolkowitz, Martin Picard, Lars Ohlsson, Francesco S Bersani, Johan Fernström, Åsa Westrin, Christina M Hough, Jue Lin, Victor I Reus, Elissa S Epel, Synthia H Mellon
Major depressive disorder (MDD) has been linked to mitochondrial defects, which could manifest in mitochondrial DNA (mtDNA) polymorphisms or mutations. Additionally, copy number of mtDNA (mtDNA-cn) can be quantified in peripheral blood mononuclear cells (PBMC)s, indirectly reflecting cellular energetics, or in the circulating cell-free mtDNA (ccf-mtDNA) levels, which may reflect a fraction of the mitochondrial genome released during cellular stress. Few studies have examined ccf-mtDNA in MDD, and no studies have tested its relationship with intracellular mtDNA-cn or with antidepressant treatment response...
January 30, 2018: Neuropsychopharmacology: Official Publication of the American College of Neuropsychopharmacology
https://www.readbyqxmd.com/read/29451229/m-aaa-proteases-mitochondrial-calcium-homeostasis-and-neurodegeneration
#13
REVIEW
Maria Patron, Hans-Georg Sprenger, Thomas Langer
The function of mitochondria depends on ubiquitously expressed and evolutionary conserved m-AAA proteases in the inner membrane. These ATP-dependent peptidases form hexameric complexes built up of homologous subunits. AFG3L2 subunits assemble either into homo-oligomeric isoenzymes or with SPG7 (paraplegin) subunits into hetero-oligomeric proteolytic complexes. Mutations in AFG3L2 are associated with dominant spinocerebellar ataxia (SCA28) characterized by the loss of Purkinje cells, whereas mutations in SPG7 cause a recessive form of hereditary spastic paraplegia (HSP7) with motor neurons of the cortico-spinal tract being predominantly affected...
February 16, 2018: Cell Research
https://www.readbyqxmd.com/read/29450720/diabetic-kidney-disease-is-there-a-role-for-glycemic-variability
#14
REVIEW
Savitha Subramanian, Irl B Hirsch
PURPOSE OF REVIEW: Diabetes is the leading cause of kidney disease globally. Diabetic kidney disease (DKD) is a heterogeneous disorder manifested as albuminuria and/or decreasing GFR. Hyperglycemic burden is the major contributor to the development of DKD. In this article, we review the evidence for the contribution of glycemic variability and the pitfalls associated with use of hemoglobin A1c (A1C), the gold standard for assessment of glucose control, in the setting of DKD. RECENT FINDINGS: Glycemic variability, characterized by swings in blood glucose levels, can result in generation of mitochondrial reactive oxygen species, a putative inciting factor for hyperglycemia-induced alterations in intracellular metabolic pathways...
February 15, 2018: Current Diabetes Reports
https://www.readbyqxmd.com/read/29449072/merrf-classification-implications-for-diagnosis-and-clinical-trials
#15
REVIEW
Josef Finsterer, Sinda Zarrouk-Mahjoub, John M Shoffner
BACKGROUND: Given the etiologic heterogeneity of disease classification using clinical phenomenology, we employed contemporary criteria to classify variants associated with myoclonic epilepsy with ragged-red fibers (MERRF) syndrome and to assess the strength of evidence of gene-disease associations. Standardized approaches are used to clarify the definition of MERRF, which is essential for patient diagnosis, patient classification, and clinical trial design. METHODS: Systematic literature and database search with application of standardized assessment of gene-disease relationships using modified Smith criteria and of variants reported to be associated with MERRF using modified Yarham criteria...
December 13, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29448258/altered-long-noncoding-rna-and-messenger-rna-expression-in-experimental-intracerebral-hemorrhage-a-preliminary-study
#16
Cui Hanjin, Liu Tao, Li Pengfei, Yang Ali, Zhou Huajun, Luo Jiekun, Wang Yang, Tang Tao
BACKGROUND/AIMS: Functional recovery in the chronic phase is a difficult problem in intracerebral hemorrhage (ICH) treatment. Long noncoding RNAs (lncRNAs) are demonstrated to be involved in central nervous system (CNS) disorders. However, the roles of lncRNAs in post-ICH injury and repair are poorly understood, especially those that may be attributed to long-term neurological deficit. The present study depicted the lncRNA and messenger RNA (mRNA) profile by microarray at late stage after an experimental ICH...
February 9, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29447225/low-apolipoprotein-a-i-levels-in-friedreich-s-ataxia-and-in-frataxin-deficient-cells-implications-for-therapy
#17
QingQing Wang, Lili Guo, Cassandra J Strawser, Lauren A Hauser, Wei-Ting Hwang, Nathaniel W Snyder, David R Lynch, Clementina Mesaros, Ian A Blair
Friedreich's ataxia (FA) is an autosomal recessive neurodegenerative disorder, which results primarily from reduced expression of the mitochondrial protein frataxin. FA has an estimated prevalence of one in 50,000 in the population, making it the most common hereditary ataxia. Paradoxically, mortality arises most frequently from cardiomyopathy and cardiac failure rather than from neurological effects. Decreased high-density lipoprotein (HDL) and apolipoprotein A-I (ApoA-l) levels in the general population are associated with an increased risk of mortality from cardiomyopathy and heart failure...
2018: PloS One
https://www.readbyqxmd.com/read/29445992/the-functional-genomics-laboratory-functional-validation-of-genetic-variants
#18
Richard J Rodenburg
Currently, one of the main challenges in human molecular genetics is the interpretation of rare genetic variants of unknown clinical significance. A conclusive diagnosis is of importance for the patient to obtain certainty about the cause of the disease, for the clinician to be able to provide optimal care to the patient and to predict the disease course, and for the clinical geneticist for genetic counseling of the patient and family members. Conclusive evidence for pathogenicity of genetic variants is therefore crucial...
February 14, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29444677/neuroprotective-effects-of-seaweeds-against-6-hydroxidopamine-induced-cell-death-on-an-in-vitro-human-neuroblastoma-model
#19
Joana Silva, Celso Alves, Susete Pinteus, Susana Mendes, Rui Pedrosa
BACKGROUND: Parkinson's disease (PD) is a progressive neurodegenerative disorder of the central nervous system. Although the causes of PD pathogenesis remain incomplete, some evidences has suggested that oxidative stress is an important mediator in its pathogenesis. The aim of this study was to evaluate the protective effects of seaweeds with high antioxidant activity on 6-hydroxydopamine (6-OHDA)-induced neurotoxicity in the human neuroblastoma cell line SH-SY5Y, as well as the associated intracellular signaling pathways...
February 14, 2018: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/29444621/excitotoxicity-in-the-pathogenesis-of-neurological-and-psychiatric-disorders-therapeutic-implications
#20
Jordi Olloquequi, Elizabeth Cornejo-Córdova, Ester Verdaguer, Francesc X Soriano, Octavio Binvignat, Carme Auladell, Antoni Camins
Neurological and psychiatric disorders are leading contributors to the global disease burden, having a serious impact on the quality of life of both patients and their relatives. Although the molecular events underlying these heterogeneous diseases remain poorly understood, some studies have raised the idea of common mechanisms involved. In excitotoxicity, there is an excessive activation of glutamate receptors by excitatory amino acids, leading to neuronal damage. Thus, the excessive release of glutamate can lead to a dysregulation of Ca 2+ homeostasis, triggering the production of free radicals and oxidative stress, mitochondrial dysfunction and eventually cell death...
February 1, 2018: Journal of Psychopharmacology
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