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Mitochondrial disease complex I

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https://www.readbyqxmd.com/read/28437736/murine-diet-induced-obesity-remodels-cardiac-and-liver-mitochondrial-phospholipid-acyl-chains-with-differential-effects-on-respiratory-enzyme-activity
#1
E Madison Sullivan, Amy Fix, Miranda J Crouch, Genevieve C Sparagna, Tonya N Zeczycki, David A Brown, Saame Raza Shaikh
Cardiac phospholipids, notably cardiolipin, undergo acyl chain remodeling and/or loss of content in aging and cardiovascular diseases, which is postulated to mechanistically impair mitochondrial function. Less is known about how diet-induced obesity influences cardiac phospholipid acyl chain composition and thus mitochondrial responses. Here we first tested if a high fat diet remodeled murine cardiac mitochondrial phospholipid acyl chain composition and consequently disrupted membrane packing, supercomplex formation and respiratory enzyme activity...
April 12, 2017: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/28434671/resveratrol-improves-mitochondrial-function-in-the-remnant-kidney-from-5-6-nephrectomized-rats
#2
Yan Hui, Miaomiao Lu, Yarong Han, Hongli Zhou, Wei Liu, Lijing Li, Ruixia Jin
Mitochondrial dysfunction is involved in the pathogenesis of chronic kidney disease (CKD). Resveratrol has been demonstrated to be beneficial for the recovery of kidney diseases. In this study, the 5/6 nephrectomized rat was used as a CKD model and the TGF-β1-exposed mouse mesangial cells were used as an in vitro model. Pathological examination showed that resveratrol treatment attenuated glomerular injury in the remnant kidney of 5/6 nephrectomized rat. Additionally, resveratrol improved mitochondrial function in vivo and in vitro, as evidenced by increasing mitochondrial membrane potential, increasing ATP, decreasing reactive oxygen species production and enhancing activities of complex I and III...
April 20, 2017: Acta Histochemica
https://www.readbyqxmd.com/read/28432755/mitochondria-and-ageing-role-in-heart-skeletal-muscle-and-adipose-tissue
#3
REVIEW
Kerstin Boengler, Maik Kosiol, Manuel Mayr, Rainer Schulz, Susanne Rohrbach
Age is the most important risk factor for most diseases. Mitochondria play a central role in bioenergetics and metabolism. In addition, several lines of evidence indicate the impact of mitochondria in lifespan determination and ageing. The best-known hypothesis to explain ageing is the free radical theory, which proposes that cells, organs, and organisms age because they accumulate reactive oxygen species (ROS) damage over time. Mitochondria play a central role as the principle source of intracellular ROS, which are mainly formed at the level of complex I and III of the respiratory chain...
April 21, 2017: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/28423196/melatonin-enhances-neural-stem-cell-differentiation-and-engraftment-by-increasing-mitochondrial-function
#4
Miguel Mendivil-Perez, Viviana Soto-Mercado, Ana Guerra-Librero, Beatriz I Fernandez-Gil, Javier Florido, Ying-Qiang Shen, Miguel A Tejada, Vivian Capilla-Gonzalez, Iryna Rusanova, José M Garcia-Verdugo, Darío Acuña-Castroviejo, Luis Carlos López, Carlos Velez-Pardo, Marlene Jimenez-Del-Rio, José M Ferrer, Germaine Escames
Neural stem cells (NSCs) are regarded as a promising therapeutic approach to protecting and restoring damaged neurons in neurodegenerative diseases (NDs) such as Parkinson's disease and Alzheimer's disease (PD and AD, respectively). However, new research suggests that NSC differentiation is required to make this strategy effective. Several studies have demonstrated that melatonin increases mature neuronal markers, which reflects NSC differentiation into neurons. Nevertheless, the possible involvement of mitochondria in the effects of melatonin during NSC differentiation has not yet been fully established...
April 19, 2017: Journal of Pineal Research
https://www.readbyqxmd.com/read/28417080/applying-a-systems-approach-to-thyroid-physiology-looking-at-the-whole-with-a-mitochondrial-perspective-instead-of-judging-single-tsh-values-or-why-we-should-know-more-about-mitochondria-to-understand-metabolism
#5
REVIEW
Roy Moncayo, Helga Moncayo
Classical thinking in endocrine physiology squeezes our diagnostic handling into a simple negative feedback mechanism with a controller and a controlled variable. In the case of the thyroid this is reduced to TSH and fT3 and fT4, respectively. The setting of this tight notion has no free space for any additions. In this paper we want to challenge this model of limited application by proposing a construct based on a systems approach departing from two basic considerations. In first place since the majority of cases of thyroid disease develop and appear during life it has to be considered as an acquired condition...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/28414167/inhibition-of-the-mitochondrial-respiratory-chain-in-gills-of-rhamdia-quelen-experimentally-infected-by-pseudomonas-aeruginosa-interplay-with-reactive-oxygen-species
#6
Matheus D Baldissera, Carine F Souza, Mateus Grings, Belisa S Parmeggiani, Guilhian Leipnitz, Karen L S Moreira, Maria Izabel U M da Rocha, Marcelo L da Veiga, Roberto C V Santos, Lenita M Stefani, Bernardo Baldisserotto
It has long been recognized that there are several infectious diseases linked to the impairment of enzymatic complexes of the mitochondrial respiratory chain, with consequent production of reactive oxygen species (ROS), that contribute to disease pathogenesis. In this study, we determined whether the inhibition on mitochondrial respiratory chain might be considered a pathway involved in the production of ROS in gills of Rhamdia quelen experimentally infected by P. aeruginosa. The animals were divided into two groups with six fish each: uninfected (the negative control group) and infected (the positive control group)...
April 13, 2017: Microbial Pathogenesis
https://www.readbyqxmd.com/read/28408307/mitochondrial-respiratory-chain-disorganization-in-parkinson-s-disease-relevant-pink1-and-dj1-mutants
#7
Irene Lopez-Fabuel, Lucia Martin-Martin, Monica Resch-Beusher, Garikoitz Azkona, Rosario Sanchez-Pernaute, Juan P Bolaños
Brain mitochondrial complex I (CI) damage is associated with the loss of the dopaminergic neurons of the Substantia Nigra in Parkinson's Disease (PD) patients. However, whether CI inhibition is associated with any alteration of the mitochondrial respiratory chain (MRC) organization in PD patients is unknown. To address this issue, here we analyzed the MRC by blue native gel electrophoresis (BNGE) followed by western blotting, in mitochondria purified from fibroblasts of patients harboring PD-relevant Pink1 mutations...
April 10, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28401997/neuroprotective-effects-of-phloretin-and-its-glycosylated-derivative-on-rotenone-induced-toxicity-in-human-sh-sy5y-neuronal-like-cells
#8
Davide Barreca, Monica Currò, Ersilia Bellocco, Silvana Ficarra, Giuseppina Laganà, Ester Tellone, Maria Laura Giunta, Giuseppa Visalli, Daniela Caccamo, Antonio Galtieri, Riccardo Ientile
Phloretin and phlorizin are the two strong natural antioxidants whose biological and pharmacological applications are rapidly growing in different human pathological conditions. The neuroprotective activity of the two flavonoids has been analyzed on cell culture of neuroblastoma cells. The neuroprotective activity of the two flavonoids has been analyzed on cell culture of neuroblastoma cells and evaluated by testing cell vitality, mitochondrial transmembrane potential and ROS production, antioxidant enzymes detection, activation of caspase 3, DNA damage, protein carbonylation, lipid peroxidation, and superoxide anion scavenging activity...
April 12, 2017: BioFactors
https://www.readbyqxmd.com/read/28385782/inhibition-of-monocarboxylate-transporter-1-by-azd3965-as-a-novel-therapeutic-approach-for-the-treatment-of-diffuse-large-b-cell-lymphoma-and-burkitt-lymphoma
#9
Richard A Noble, Natalie Bell, Helen Blair, Arti Sikka, Huw Thomas, Nicole Phillips, Sirintra Nakjang, Satomi Miwa, Rachel Crossland, Vikki Rand, Despina Televantou, Anna Long, Hector C Keun, Chris M Bacon, Simon Bomken, Susan E Critchlow, Stephen R Wedge
Inhibition of monocarboxylate transporter 1 has been proposed as a therapeutic approach to perturb lactate shuttling in tumor cells that lack monocarboxylate transporter 4. We examined the monocarboxylate transporter 1 inhibitor AZD3965, currently in phase I clinical studies, as a potential therapy for diffuse large B-cell lymphoma and Burkitt lymphoma. Whilst extensive monocarboxylate transporter 1 protein was found in 120 diffuse large B-cell lymphoma and 10 Burkitt lymphoma patient tumors, monocarboxylate transporter 4 protein expression was undetectable in 73% of the diffuse large B-cell lymphoma samples and undetectable or negligible in each Burkitt lymphoma sample...
April 6, 2017: Haematologica
https://www.readbyqxmd.com/read/28379402/slp-2-interacts-with-parkin-in-mitochondria-and-prevents-mitochondrial-dysfunction-in-parkin-deficient-human-ipsc-derived-neurons-and-drosophila
#10
Alessandra Zanon, Sreehari Kalvakuri, Aleksandar Rakovic, Luisa Foco, Marianna Guida, Christine Schwienbacher, Alice Serafin, Franziska Rudolph, Michaela Trilck, Anne Grünewald, Nancy Stanslowsky, Florian Wegner, Valentina Giorgio, Alexandros A Lavdas, Rolf Bodmer, Peter P Pramstaller, Christine Klein, Andrew A Hicks, Irene Pichler, Philip Seibler
Mutations in the Parkin gene (PARK2) have been linked to a recessive form of Parkinson's disease (PD) characterized by the loss of dopaminergic neurons in the substantia nigra. Deficiencies of mitochondrial respiratory chain complex I activity have been observed in the substantia nigra of PD patients, and loss of Parkin results in the reduction of complex I activity shown in various cell and animal models. Using co-immunoprecipitation and proximity ligation assays on endogenous proteins, we demonstrate that Parkin interacts with mitochondrial Stomatin-like protein 2 (SLP-2), which also binds the mitochondrial lipid cardiolipin and functions in the assembly of respiratory chain proteins...
April 3, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28372990/neurotoxic-mechanisms-by-which-the-usp14-inhibitor-iu1-depletes-ubiquitinated-proteins-and-tau-in-rat-cerebral-cortical-neurons-relevance-to-alzheimer-s-disease
#11
Magdalena J Kiprowska, Anna Stepanova, Dustin R Todaro, Alexander Galkin, Arthur Haas, Scott M Wilson, Maria E Figueiredo-Pereira
In Alzheimer's disease proteasome activity is reportedly downregulated, thus increasing it could be therapeutically beneficial. The proteasome-associated deubiquitinase USP14 disassembles polyubiquitin-chains, potentially delaying proteasome-dependent protein degradation. We assessed the protective efficacy of inhibiting or downregulating USP14 in rat and mouse (Usp14(axJ)) neuronal cultures treated with prostaglandin J2 (PGJ2). IU1 concentrations (HIU1>25μM) reported by others to inhibit USP14 and be protective in non-neuronal cells, reduced PGJ2-induced Ub-protein accumulation in neurons...
March 31, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28368682/stress-dependent-opposing-roles-for-mitophagy-in-aging-of-the-ascomycete-podospora-anserina
#12
Laura Knuppertz, Verena Warnsmann, Andrea Hamann, Carolin Grimm, Heinz D Osiewacz
Mitochondrial dysfunction is causatively linked to organismal aging and the development of degenerative diseases. Here we describe stress-dependent opposing roles of mitophagy, the selective autophagic degradation of mitochondria, in aging and life-span control. We report that the ablation of the mitochondrial superoxide dismutase which is involved in reactive oxygen species (ROS) balancing, does not affect life span of the fungal aging model Podospora anserina, although superoxide levels are strongly increased and complex I-dependent respiration is impaired...
April 3, 2017: Autophagy
https://www.readbyqxmd.com/read/28350990/the-putative-drp1-inhibitor-mdivi-1-is-a-reversible-mitochondrial-complex-i-inhibitor-that-modulates-reactive-oxygen-species
#13
Evan A Bordt, Pascaline Clerc, Brian A Roelofs, Andrew J Saladino, László Tretter, Vera Adam-Vizi, Edward Cherok, Ahmed Khalil, Nagendra Yadava, Shealinna X Ge, T Chase Francis, Nolan W Kennedy, Lora K Picton, Tanya Kumar, Sruti Uppuluri, Alexandrea M Miller, Kie Itoh, Mariusz Karbowski, Hiromi Sesaki, R Blake Hill, Brian M Polster
Mitochondrial fission mediated by the GTPase dynamin-related protein 1 (Drp1) is an attractive drug target in numerous maladies that range from heart disease to neurodegenerative disorders. The compound mdivi-1 is widely reported to inhibit Drp1-dependent fission, elongate mitochondria, and mitigate brain injury. Here, we show that mdivi-1 reversibly inhibits mitochondrial complex I-dependent O2 consumption and reverse electron transfer-mediated reactive oxygen species (ROS) production at concentrations (e...
March 27, 2017: Developmental Cell
https://www.readbyqxmd.com/read/28348546/dementia-with-lewy-bodies-molecular-pathology-in-the-frontal-cortex-in-typical-and-rapidly-progressive-forms
#14
Paula Garcia-Esparcia, Irene López-González, Oriol Grau-Rivera, María Francisca García-Garrido, Anusha Konetti, Franc Llorens, Saima Zafar, Margarita Carmona, José Antonio Del Rio, Inga Zerr, Ellen Gelpi, Isidro Ferrer
OBJECTIVES: The goal of this study was to assess mitochondrial function, energy, and purine metabolism, protein synthesis machinery from the nucleolus to the ribosome, inflammation, and expression of newly identified ectopic olfactory receptors (ORs) and taste receptors (TASRs) in the frontal cortex of typical cases of dementia with Lewy bodies (DLB) and cases with rapid clinical course (rpDLB: 2 years or less) compared with middle-aged non-affected individuals, in order to learn about the biochemical abnormalities underlying Lewy body pathology...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28335035/a-novel-cisd2-mutation-associated-with-a-classical-wolfram-syndrome-phenotype-alters-ca2-homeostasis-and-er-mitochondria-interactions
#15
Cécile Rouzier, David Moore, Cécile Delorme, Sandra Lacas-Gervais, Samira Ait-El-Mkadem, Konstantina Fragaki, Florence Burté, Valérie Serre, Sylvie Bannwarth, Annabelle Chaussenot, Martin Catala, Patrick Yu-Wai-Man, Véronique Paquis-Flucklinger
Wolfram syndrome (WS) is progressive neurodegenerative disease characterized by early-onset optic atrophy and diabetes mellitus, which can be associated with more extensive central nervous system and endocrine complications. The majority of patients harbour pathogenic WFS1 mutations, but recessive mutations in a second gene, CISD2, have been described in a small number of families with Wolfram syndrome type 2 (WFS2). The defining diagnostic criteria for WFS2 also consist of optic atrophy and diabetes mellitus, but unlike WFS1, this phenotypic subgroup has been associated with peptic ulcer disease and an increased bleeding tendency...
March 6, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28327638/conditional-deletion-of-ndufs4-in-dopaminergic-neurons-promotes-parkinson-s-disease-like-non-motor-symptoms-without-loss-of-dopamine-neurons
#16
Won-Seok Choi, Hyung-Wook Kim, François Tronche, Richard D Palmiter, Daniel R Storm, Zhengui Xia
Reduction of mitochondrial complex I activity is one of the major hypotheses for dopaminergic neuron death in Parkinson's disease. However, reduction of complex I activity in all cells or selectively in dopaminergic neurons via conditional deletion of the Ndufs4 gene, a subunit of the mitochondrial complex I, does not cause dopaminergic neuron death or motor impairment. Here, we investigated the effect of reduced complex I activity on non-motor symptoms associated with Parkinson's disease using conditional knockout (cKO) mice in which Ndufs4 was selectively deleted in dopaminergic neurons (Ndufs4 cKO)...
March 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28287425/the-relationship-between-mitochondrial-respiratory-chain-activities-in-muscle-and-metabolites-in-plasma-and-urine-a-retrospective-study
#17
Corinne Alban, Elena Fatale, Abed Joulani, Polina Ilin, Ann Saada
The relationship between 114 cases with decreased enzymatic activities of mitochondrial respiratory chain (MRC) complexes I-V (C I-V) in muscle and metabolites in urine and plasma was retrospectively examined. Less than 35% disclosed abnormal plasma amino acids and acylcarnitines, with elevated alanine and low free carnitine or elevated C4-OH-carnitine as the most common findings, respectively. Abnormal urine organic acids (OA) were detected in 82% of all cases. In CI and CII defects, lactic acid (LA) in combination with other metabolites was the most common finding...
March 10, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28279569/lifetime-exercise-intolerance-with-lactic-acidosis-as-key-manifestation-of-novel-compound-heterozygous-acad9-mutations-causing-complex-i-deficiency
#18
Bertold Schrank, Benedikt Schoser, Thomas Klopstock, Peter Schneiderat, Rita Horvath, Angela Abicht, Elke Holinski-Feder, Sarunas Augustis
We report a 36-year-old female having lifetime exercise intolerance and lactic acidosis with nausea associated with novel compound heterozygous Acyl-CoA dehydrogenase 9 gene (ACAD9) mutations (p.Ala390Thr and p.Arg518Cys). ACAD9 is an assembly factor for the mitochondrial respiratory chain complex I. ACAD9 mutations are recognized as frequent causes of complex I deficiency. Our patient presented with exercise intolerance, rapid fatigue, and nausea since early childhood. Mild physical workload provoked the occurrence of nausea and vomiting repeatedly...
February 14, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28274885/molecular-identification-of-wild-triatomines-of-the-genus-rhodnius-in-the-bolivian-amazon-strategy-and-current-difficulties
#19
Simone Frédérique Brenière, Edwin Wily Condori, Rosio Buitrago, Luis Fernando Sosa, Catarina Lopes Macedo, Christian Barnabé
The Amazon region has recently been considered as endemic in Latin America. In Bolivia, the vast Amazon region is undergoing considerable human migrations and substantial anthropization of the environment, potentially renewing the danger of establishing the transmission of Chagas disease. The cases of human oral contamination occurring in 2010 in the town of Guayaramerín provided reasons to intensify research. As a result, the goal of this study was to characterize the species of sylvatic triatomines circulating in the surroundings of Yucumo (Beni, Bolivia), a small Amazonian city at the foot of the Andes between the capital (La Paz) and Trinidad the largest city of Beni...
March 6, 2017: Infection, Genetics and Evolution
https://www.readbyqxmd.com/read/28273955/lack-of-xpc-leads-to-a-shift-between-respiratory-complexes-i-and-ii-but-sensitizes-cells-to-mitochondrial-stress
#20
Mateus P Mori, Rute A P Costa, Daniela T Soltys, Thiago de S Freire, Franco A Rossato, Ignácio Amigo, Alicia J Kowaltowski, Aníbal E Vercesi, Nadja C de Souza-Pinto
Genomic instability drives tumorigenesis and DNA repair defects are associated with elevated cancer. Metabolic alterations are also observed during tumorigenesis, although a causal relationship between these has not been clearly established. Xeroderma pigmentosum (XP) is a DNA repair disease characterized by early cancer. Cells with reduced expression of the XPC protein display a metabolic shift from OXPHOS to glycolysis, which was linked to accumulation of nuclear DNA damage and oxidants generation via NOX-1...
December 2017: Scientific Reports
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