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OXPHOS disease

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https://www.readbyqxmd.com/read/29729355/insertion-of-proteolipid-protein-into-mitochondria-but-not-dm20-regulates-metabolism-of-cells
#1
Mallika Somayajulu, Denise A Bessert, Maik Hüttemann, Jasloveleen Sohi, John Kamholz, Robert P Skoff
Proteolipid protein (PLP), besides its adhesive role in myelin, has been postulated to have multiple cellular functions. One well-documented function of PLP is regulation of oligodendrocyte (Olg) apoptosis. In contrast, DM20, an alternatively spliced product of the PLP1/Plp1 gene, has been proposed to have functions that are unique from PLP but these functions have never been elucidated. Here, we compare metabolism of PLP and DM20, and show that oxidative phosphorylation (OxPhos) was significantly decreased in Plp1 but not DM20 or EGFP expressing cells...
May 2, 2018: Neuroscience Letters
https://www.readbyqxmd.com/read/29723585/platycodon-grandiflorum-derived-saponin-enhances-exercise-function-skeletal-muscle-protein-synthesis-and-mitochondrial-function
#2
Yong An Kim, Sun Woo Jin, Suck Hoon Oh, Gi Ho Lee, Hoa Thi Pham, Jae Ho Choi, Young Chul Chung, Wang Lok Lee, Sang Kyum Kim, Hye Gwang Jeong
Lower physical performance is an important risk factor in hypokinetic-related chronic disease, metabolic syndrome, and muscle atrophy. Our previous research demonstrated that Platycodon grandiflorum-derived saponin (PS) protects against eccentric exercise-induced muscle damage and mitochondrial function-related peroxisomal acyl-coenzme A oxidase (ACOX-1) and carnitine palmitoyltransferase (CPT-1) in high-fat diet-induced non-alcoholic steatohepatitis, and it inhibits osteoclast differentiation. However, the effects of PS on physical performance remain unknown...
April 30, 2018: Food and Chemical Toxicology
https://www.readbyqxmd.com/read/29700325/kh176-safeguards-mitochondrial-diseased-cells-from-redox-stress-induced-cell-death-by-interacting-with-the-thioredoxin-system-peroxiredoxin-enzyme-machinery
#3
Julien Beyrath, Mina Pellegrini, Herma Renkema, Lisanne Houben, Svetlana Pecheritsyna, Peter van Zandvoort, Petra van den Broek, Akkiz Bekel, Pierre Eftekhari, Jan A M Smeitink
A deficient activity of one or more of the mitochondrial oxidative phosphorylation (OXPHOS) enzyme complexes leads to devastating diseases, with high unmet medical needs. Mitochondria, and more specifically the OXPHOS system, are the main cellular production sites of Reactive Oxygen Species (ROS). Increased ROS production, ultimately leading to irreversible oxidative damage of macromolecules or to more selective and reversible redox modulation of cell signalling, is a causative hallmark of mitochondrial diseases...
April 26, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29700116/comparative-analysis-of-parkinson-s-disease-associated-genes-reveals-altered-survival-and-bioenergetics-of-parkin-deficient-dopamine-neurons-in-mice
#4
Nicolas Giguere, Consiglia Pacelli, Caroline Saumure, Marie-Josee Bourque, Diana Matheoud, Daniel Levesque, Ruth S Slack, David S Park, Louis-Eric Trudeau
Many mutations in genes encoding proteins such as parkin, PTEN-induced putative kinase 1 (PINK1), protein deglycase DJ-1 (DJ-1 or PARK7), leucine-rich repeat kinase 2 (LRRK2), and α-synuclein have been linked to familial forms of Parkinson's disease (PD). The consequences of these mutations, such as altered mitochondrial function and pathological protein aggregation, are starting to be better understood. However, little is known about the mechanisms explaining why alterations in such diverse cellular mechanisms lead to the selective loss of dopamine (DA) neurons in the substantia nigra (SNc) in the brain of individuals with PD...
April 26, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29683527/modulation-of-allergic-responses-by-mitochondrial-stat3-inhibitors
#5
Tal Erlich, Israa Sharkia, Nadine Landolina, Miri Assayag, Omer Goldberger, Neville Berkman, Francesca Levi-Schafer, Ehud Razin
Recently we have shown that mast cell mitochondrial STAT3 could serve as a new target for the regulation of the allergic response as it plays an essential role in immunologically-mediated degranulation of mast cells. In the present work, we explored how two recently developed mitochondrial STAT3 inhibitors (Mitocur-1 and Mitocur-3) modulate the allergic response. Here we show that treatment of rodent and human cultured mast cells with low concentrations of mitochondrial STAT3 inhibitors had no effect on STAT3 target gene expression...
April 23, 2018: Allergy
https://www.readbyqxmd.com/read/29664677/silac-based-quantitative-proteomic-analysis-of-the-livers-of-spontaneous-obese-and-diabetic-rhesus-monkeys
#6
Junlong Wang, Shimeng Xu, Jing Gao, Linqiang Zhang, Zhiguo Zhang, Wenhui Yang, Yunhai Li, Shasha Liao, Hu Zhou, Pingsheng Liu, Bin Liang
Type 2 diabetes mellitus (T2DM) is a severely metabolic disorder that affects above 10% worldwide population. Obesity is a major cause of insulin resistance and contributes to the development of T2DM. Liver is an essential metabolic organ that plays crucial roles in the pathogenesis of obesity and diabetes. However, the underlying mechanisms of liver in the transition of obesity to diabetes are not fully understood. Nonhuman primate (NHP) rhesus monkey is an appropriate animal for research of human diseases...
April 17, 2018: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29656361/characterization-of-the-mitochondrial-aerobic-metabolism-in-the-pre-and-perisynaptic-districts-of-the-sod1-g93a-mouse-model-of-amyotrophic-lateral-sclerosis
#7
Silvia Ravera, Tiziana Bonifacino, Martina Bartolucci, Marco Milanese, Elena Gallia, Francesca Provenzano, Katia Cortese, Isabella Panfoli, Giambattista Bonanno
Amyotrophic lateral sclerosis (ALS) is an adult-onset fatal neurodegenerative disease characterized by muscle wasting, weakness, and spasticity due to a progressive degeneration of cortical, brainstem, and spinal motor neurons. The etiopathological causes are still largely obscure, although astrocytes definitely play a role in neuronal damage. Several mechanisms have been proposed to concur to neurodegeneration in ALS, including mitochondrial dysfunction. We have previously shown profound modifications of glutamate release and presynaptic plasticity in the spinal cord of the SOD1 G93A mouse model of ALS...
April 14, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29655867/short-term-sleep-deprivation-with-exposure-to-nocturnal-light-alters-mitochondrial-bioenergetics-in-drosophila
#8
Nathane Rosa Rodrigues, Giulianna Echeverria Macedo, Illana Kemmerich Martins, Karen Kich Gomes, Nélson Rodrigues de Carvalho, Thaís Posser, Jeferson Luis Franco
Many studies have shown the effects of sleep deprivation in several aspects of health and disease. However, little is known about how mitochondrial bioenergetics function is affected under this condition. To clarify this, we developed a simple model of short-term sleep deprivation, in which fruit-flies were submitted to a nocturnal light condition and then mitochondrial parameters were assessed by high resolution respirometry (HRR). Exposure of flies to constant light was able to alter sleep patterns, causing locomotor deficits, increasing ROS production and lipid peroxidation, affecting mitochondrial activity, antioxidant defense enzymes and caspase activity...
April 12, 2018: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/29655782/lithocholic-acid-a-bacterial-metabolite-reduces-breast-cancer-cell-proliferation-and-aggressiveness
#9
Edit Mikó, András Vida, Tünde Kovács, Gyula Ujlaki, György Trencsényi, Judit Márton, Zsanett Sári, Patrik Kovács, Anita Boratkó, Zoltán Hujber, Tamás Csonka, Péter Antal-Szalmás, Mitsuhiro Watanabe, Imre Gombos, Balazs Csoka, Borbála Kiss, László Vígh, Judit Szabó, Gábor Méhes, Anna Sebestyén, James J Goedert, Péter Bai
Our study aimed at finding a mechanistic relationship between the gut microbiome and breast cancer. Breast cancer cells are not in direct contact with these microbes, but disease could be influenced by bacterial metabolites including secondary bile acids that are exclusively synthesized by the microbiome and known to enter the human circulation. In murine and bench experiments, a secondary bile acid, lithocholic acid (LCA), reduced cancer cell proliferation (by 10-20%) and VEGF production (by 37%), aggressiveness and metastatic potential of primary tumors through inducing mesenchymal-to-epithelial transition, increased antitumor immune response, OXPHOS and the TCA cycle...
April 12, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29618761/qil1-dependent-assembly-of-micos-complex-lethal-mutation-in-c19orf70-resulting-in-liver-disease-and-severe-neurological-retardation
#10
J Gödiker, M Grüneberg, I DuChesne, J Reunert, S Rust, C Westermann, Y Wada, G Classen, C D Langhans, K P Schlingmann, R J Rodenburg, R Pohlmann, T Marquardt
Seven subunits of the mitochondrial contact site and cristae junction (CJ) organizing system (MICOS) in humans have been recently described in function and structure. QIL1 (also named MIC13) is a small complex that is crucial for the maintenance and assembling of MICOS. A novel mutation of an essential splice site in the C19orf70 gene encoding QIL1 induces severe mitochondrial encephalopathy, hepatopathy and lactate acidosis consistent with psychomotor retardation. In addition, bilateral kidney stones were observed...
April 4, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29593495/mitochondrial-molecular-abnormalities-revealed-by-proteomic-analysis-of-hippocampal-organelles-of-mice-triple-transgenic-for-alzheimer-disease
#11
Haitao Yu, Xuemei Lin, Dian Wang, Zaijun Zhang, Yi Guo, Xiaohu Ren, Benhong Xu, Jianhui Yuan, Jianjun Liu, Peter S Spencer, Jian-Zhi Wang, Xifei Yang
Mitochondrial dysfunction is implicated in the pathogenesis of Alzheimer's disease (AD). However, the precise mitochondrial molecular deficits in AD remain poorly understood. Mitochondrial and nuclear proteomic analysis in mature male triple transgenic AD mice (PS1M146V/APPSwe/TauP301L) by two-dimensional fluorescence difference gel electrophoresis (2D-DIGE) coupled with MALDI-TOF-MS/MS, bio-informatics analysis and immunofluorescent staining were performed in this study. In addition to impaired spatial memory impairment and intracellular accumulation of amyloid 1-42 (Aβ1-42 ) in the 3xTg-AD mice, a well-accepted mouse model of the human disease, we also found significantly increased DNA oxidative damage in entorhinal cortex, hippocampal CA1, CA3 and dental gyrus (DG), as evidenced by the positive staining of 8-hydroxyguanosine, a biomarker of mild cognitive impairment early in AD...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29576219/bi-allelic-mutations-in-the-mitochondrial-ribosomal-protein-mrps2-cause-sensorineural-hearing-loss-hypoglycemia-and-multiple-oxphos-complex-deficiencies
#12
Thatjana Gardeitchik, Miski Mohamed, Benedetta Ruzzenente, Daniela Karall, Sergio Guerrero-Castillo, Daisy Dalloyaux, Mariël van den Brand, Sanne van Kraaij, Ellyze van Asbeck, Zahra Assouline, Marlene Rio, Pascale de Lonlay, Sabine Scholl-Buergi, David F G J Wolthuis, Alexander Hoischen, Richard J Rodenburg, Wolfgang Sperl, Zsolt Urban, Ulrich Brandt, Johannes A Mayr, Sunnie Wong, Arjan P M de Brouwer, Leo Nijtmans, Arnold Munnich, Agnès Rötig, Ron A Wevers, Metodi D Metodiev, Eva Morava
Biogenesis of the mitochondrial oxidative phosphorylation system, which produces the bulk of ATP for almost all eukaryotic cells, depends on the translation of 13 mtDNA-encoded polypeptides by mitochondria-specific ribosomes in the mitochondrial matrix. These mitoribosomes are dual-origin ribonucleoprotein complexes, which contain mtDNA-encoded rRNAs and tRNAs and ∼80 nucleus-encoded proteins. An increasing number of gene mutations that impair mitoribosomal function and result in multiple OXPHOS deficiencies are being linked to human mitochondrial diseases...
April 5, 2018: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29571252/hepatoprotective-effects-of-capsaicin-and-alpha-tocopherol-on-mitochondrial-function-in-mice-fed-a-high-fat-diet
#13
Vedat Ekero Lu, Birsen Ayd N, Zülal Atl Ekero Lu, Yasemin Özdener Kömpe
Capsaicin (CAP) and alpha-tocopherol (TOC) have antioxidant properties. We investigated effects of CAP and TOC on mitochondrial oxidative stress and mitochondrial bioenergetics in liver of mice fed HFD. AST, ALT, glucose, homeostasis model assessment-insulin resistance index ((HOMA-IR)) and mitochondrial oxidative stress parameters increased, whereas oxidative phosphorylation (OXPHOS) enzymes, tricarboxylic acid cycle (TCA) enzymes, ATP level and mitochondrial metabolic function (MTT) decreased in mice fed a HFD compared to the fed a standard diet (NC)...
February 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29547561/targeting-mitochondria-to-counteract-age-related-cellular-dysfunction
#14
REVIEW
Corina T Madreiter-Sokolowski, Armin A Sokolowski, Markus Waldeck-Weiermair, Roland Malli, Wolfgang F Graier
Senescence is related to the loss of cellular homeostasis and functions, which leads to a progressive decline in physiological ability and to aging-associated diseases. Since mitochondria are essential to energy supply, cell differentiation, cell cycle control, intracellular signaling and Ca2+ sequestration, fine-tuning mitochondrial activity appropriately, is a tightrope walk during aging. For instance, the mitochondrial oxidative phosphorylation (OXPHOS) ensures a supply of adenosine triphosphate (ATP), but is also the main source of potentially harmful levels of reactive oxygen species (ROS)...
March 16, 2018: Genes
https://www.readbyqxmd.com/read/29514047/deletion-of-nlrx1-increases-fatty-acid-metabolism-and-prevents-diet-induced-hepatic-steatosis-and-metabolic-syndrome
#15
Lotte Kors, Elena Rampanelli, Geurt Stokman, Loes M Butter, Ntsiki M Held, Nike Claessen, Per W B Larsen, Joanne Verheij, Coert J Zuurbier, Gerhard Liebisch, Gerd Schmitz, Stephen E Girardin, Sandrine Florquin, Riekelt H Houtkooper, Jaklien C Leemans
NOD-like receptor (NLR)X1 (NLRX1) is an ubiquitously expressed inflammasome-independent NLR that is uniquely localized in mitochondria with as yet unknown effects on metabolic diseases. Here, we report that NLRX1 is essential in regulating cellular metabolism in non-immune parenchymal hepatocytes by decreasing mitochondrial fatty acid-dependent oxidative phosphorylation (OXPHOS) and promoting glycolysis. NLRX1 loss in mice has a profound impact on the prevention of diet-induced metabolic syndrome parameters, non-alcoholic fatty liver disease (NAFLD) progression, and renal dysfunction...
May 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29505781/analyzing-mitochondrial-function-in-human-peripheral-blood-mononuclear-cells
#16
Chao-Pin Hsiao, Charles Hoppel
Mitochondrial oxidative phosphorylation (OXPHOS) is responsible for producing most of the adenosine triphosphate required by eukaryotic cells. Lymphocytes make up the majority of the peripheral blood mononuclear cells. Peripheral blood mononuclear cells are readily obtainable, providing an ideal sample to monitor systemic changes and understand molecular signaling mechanisms in disease processes. Mitochondrial energy metabolism of lymphocyte has been used to screen for OXPHOS disorders. While there are increasing studies of lymphocyte OXPHOS, few studies examined activity of electron transport chain of lymphocyte mitochondria...
May 15, 2018: Analytical Biochemistry
https://www.readbyqxmd.com/read/29500454/the-effects-of-angiotensin-receptor-neprilysin-inhibition-by-sacubitril-valsartan-on-adipose-tissue-transcriptome-and-protein-expression-in-obese-hypertensive-patients
#17
R Stinkens, B W van der Kolk, J Jordan, T Jax, S Engeli, T Heise, J W Jocken, M May, C Schindler, B Havekes, N Schaper, D Albrecht, S Kaiser, N Hartmann, M Letzkus, T H Langenickel, G H Goossens, E E Blaak
Increased activation of the renin-angiotensin system is involved in the onset and progression of cardiometabolic diseases, while natriuretic peptides (NP) may exert protective effects. We have recently demonstrated that sacubitril/valsartan (LCZ696), a first-in-class angiotensin receptor neprilysin inhibitor, which blocks the angiotensin II type-1 receptor and augments natriuretic peptide levels, improved peripheral insulin sensitivity in obese hypertensive patients. Here, we investigated the effects of sacubitril/valsartan (400 mg QD) treatment for 8 weeks on the abdominal subcutaneous adipose tissue (AT) phenotype compared to the metabolically neutral comparator amlodipine (10 mg QD) in 70 obese hypertensive patients...
March 2, 2018: Scientific Reports
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
#18
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...
May 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29440775/genetic-defects-in-mtdna-encoded-protein-translation-cause-pediatric-mitochondrial-cardiomyopathy-with-early-onset-brain-disease
#19
Rick Kamps, Radek Szklarczyk, Tom E Theunissen, Debby M E I Hellebrekers, Suzanne C E H Sallevelt, Iris B Boesten, Bart de Koning, Bianca J van den Bosch, Gajja S Salomons, Marisa Simas-Mendes, Rob Verdijk, Kees Schoonderwoerd, Irenaeus F M de Coo, Jo M Vanoevelen, Hubert J M Smeets
This study aims to identify gene defects in pediatric cardiomyopathy and early-onset brain disease with oxidative phosphorylation (OXPHOS) deficiencies. We applied whole-exome sequencing in three patients with pediatric cardiomyopathy and early-onset brain disease with OXPHOS deficiencies. The brain pathology was studied by MRI analysis. In consanguineous patient 1, we identified a homozygous intronic variant (c.850-3A > G) in the QRSL1 gene, which was predicted to cause abnormal splicing. The variant segregated with the disease and affected the protein function, which was confirmed by complementation studies, restoring OXPHOS function only with wild-type QRSL1...
April 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29433569/mh84-improves-mitochondrial-dysfunction-in-a-mouse-model-of-early-alzheimer-s-disease
#20
Maximilian Pohland, Maren Pellowska, Heike Asseburg, Stephanie Hagl, Martina Reutzel, Aljoscha Joppe, Dirk Berressem, Schamim H Eckert, Mario Wurglics, Manfred Schubert-Zsilavecz, Gunter P Eckert
BACKGROUND: Current approved drugs for Alzheimer's disease (AD) only attenuate symptoms, but do not cure the disease. The pirinixic acid derivate MH84 has been characterized as a dual gamma-secretase/proliferator activated receptor gamma (PPARγ) modulator in vitro. Pharmacokinetic studies in mice showed that MH84 is bioavailable after oral administration and reaches the brain. We recently demonstrated that MH84 improved mitochondrial dysfunction in a cellular model of AD. In the present study, we extended the pharmacological characterization of MH84 to 3-month-old Thy-1 AβPPSL mice (harboring the Swedish and London mutation in human amyloid precursor protein (APP)) which are characterized by enhanced AβPP processing and cerebral mitochondrial dysfunction, representing a mouse model of early AD...
February 13, 2018: Alzheimer's Research & Therapy
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