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Succinate dehydrogenase

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https://www.readbyqxmd.com/read/27910947/renal-cell-carcinoma-with-tfe3-translocation-and-succinate-dehydrogenase-b-mutation
#1
Anna Calió, David J Grignon, Bradley A Stohr, Sean R Williamson, John N Eble, Liang Cheng
Translocation renal cell carcinoma and succinate dehydrogenase (SDH)-deficient renal cell carcinoma are now recognized as specific renal tumor types in the World Health Organization (WHO) classification. Both have limited immunohistochemical positivity for epithelial markers, and the spectrum of morphology continues to widen for both of these entities. We identified four renal cell carcinomas with positive TFE3 immunohistochemical staining and negative SDHB staining. The patients (2F, 2M) ranged in age from 19 to 65 years...
December 2, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27909949/organ-specific-effects-of-low-dose-zinc-pre-exposure-on-high-dose-zinc-induced-mitochondrial-dysfunction-in-large-yellow-croaker-pseudosciaena-crocea
#2
Jia-Lang Zheng, Shuang-Shuang Yuan, Bin Shen, Chang-Wen Wu
The study was carried out to evaluate the effects of low-dose zinc (Zn) pre-exposure on survival rate, new Zn accumulation, and mitochondrial bioenergetics in the liver and spleen of large yellow croaker exposed to high-dose Zn. To the end, fish were pre-exposed to 0 and 2 mg L(-1) Zn for 48 h and post-exposed to 0 and 12 mg L(-1) Zn for 48 h. Twelve milligrams Zn per liter exposure alone reduced survival rate, but the effect did not appear in the 2 mg L(-1) Zn pre-exposure groups. Two milligrams per liter Zn pre-exposure also ameliorated 12 mg Zn L(-1) induced new Zn accumulation, reactive oxygen species (ROS) levels, and mitochondrial swelling in the liver...
December 1, 2016: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/27909649/in-silico-profiling-of-cell-growth-and-succinate-production-in-escherichia-coli-nzn111
#3
Xingxing Jian, Ningchuan Li, Cheng Zhang, Qiang Hua
BACKGROUND: Succinic acid is a valuable product due to its wide-ranging utilities. To improve succinate production and reduce by-products formation, Escherichia coli NZN111 was constructed by insertional inactivation of lactate dehydrogenase (LDH) and pyruvate formate lyase (PFL) encoded by the genes ldhA and pflB, respectively. However, this double-deletion mutant is incapable of anaerobically growing on glucose in rich or minimal medium even with acetate supplementation. A widespread hold view is that the inactivation of NADH-dependent LDH limits the regeneration of NAD(+) and consequently disables proper growth under anaerobic conditions...
2016: Bioresources and Bioprocessing
https://www.readbyqxmd.com/read/27908774/increased-incorporation-of-gaseous-co2-into-succinate-by-escherichia-coli-overexpressing-carbonic-anhydrase-and-phosphoenolpyruvate-carboxylase-genes
#4
Soohyun Park, Jae-Ung Lee, Sukhyeong Cho, Hyeonsoo Kim, Han Bin Oh, Seung Pil Pack, Jinwon Lee
Carbon dioxide (CO2) is an abundant and cheap carbon source that is partly responsible for global warming in the atmosphere. The objective of this study was to construct a recombinant E. coli strain that can show enhanced production of succinate derived from CO2. In this study, we confirmed the enhancement of utilization by analyzing succinate containing one carbon-13 ((13)C) derived from (13)CO2. Firstly, the carbonic anhydrase gene (SP(-)HCCA) derived from Hahella chejuensis KCTC 2396 was over-expressed to enhance carbon flux toward bicarbonate ion (HCO3(-)) synthesis in E...
November 28, 2016: Journal of Biotechnology
https://www.readbyqxmd.com/read/27908708/chlorogenic-acid-ameliorates-isoproterenol-induced-myocardial-injury-in-rats-by-stabilizing-mitochondrial-and-lysosomal-enzymes
#5
Palaniyandi Akila, Lourthurani Asaikumar, Lakshmanan Vennila
This study was deliberated to aspire the effects of chlorogenic acid (CGA) against myocardial infarction (MI) induced by Isoproterenol (ISO), in a rat model. In the pathology of MI, enzymes released due to the mitochondrial and lysosomal lipid peroxidation play an integral role. Induction of rats with ISO (85mg/kg BW) for 2 consecutive days resulted in a significant decrease in the activities of heart mitochondrial enzymes isocitrate dehydrogenase (ICDH), α-ketoglutarate dehydrogenase (α-KGDH), succinate dehydrogenase (SDH) and malate dehydrogenase (MDH)...
November 28, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27906110/failed-upregulation-of-tfam-protein-and-mitochondrial-dna-in-oxidatively-deficient-fibers-of-chronic-obstructive-pulmonary-disease-locomotor-muscle
#6
Yana Konokhova, Sally Spendiff, R Thomas Jagoe, Sudhakar Aare, Sophia Kapchinsky, Norah J MacMillan, Paul Rozakis, Martin Picard, Mylène Aubertin-Leheudre, Charlotte H Pion, Jean Bourbeau, Russell T Hepple, Tanja Taivassalo
BACKGROUND: Low mitochondrial content and oxidative capacity are well-established features of locomotor muscle dysfunction, a prevalent and debilitating systemic occurrence in patients with chronic obstructive pulmonary disease (COPD). Although the exact cause is not firmly established, physical inactivity and oxidative stress are among the proposed underlying mechanisms. Here, we assess the impact of COPD pathophysiology on mitochondrial DNA (mtDNA) integrity, biogenesis, and cellular oxidative capacity in locomotor muscle of COPD patients and healthy controls...
February 18, 2016: Skeletal Muscle
https://www.readbyqxmd.com/read/27905048/loss-of-succinate-dehydrogenase-subunit-b-sdhb-as-a-prognostic-factor-in-advanced-ileal-well-differentiated-neuroendocrine-tumors
#7
Massimo Milione, Patrick Maisonneuve, Alessio Pellegrinelli, Sara Pusceddu, Giovanni Centonze, Francesca Dominoni, Cecilia Brambilla, Manila Rubino, Antongiulio Faggiano, Roberto Buzzoni, Laura Concas, Luca Giacomelli, Jorgelina Coppa, Vincenzo Mazzaferro, Filippo de Braud
PURPOSE: Abnormal expression of succinate dehydrogenase, (SDH), in particular of the B subunit (SDHB), is implicated in the pathogenesis of neuroendocrine tumors. This study evaluates the distribution of SDHB in WHO grading G1 and G2 intestinal, well-differentiated neuroendocrine tumors and corresponding lymph node or liver metastases. METHODS: We collected ileal well-differentiated neuroendocrine tumors specimens from consecutive patients with prior primary resection and distant synchronous or metachronous liver metastases...
November 30, 2016: Endocrine
https://www.readbyqxmd.com/read/27900521/-18-f-fluorodihydroxyphenylalanine-pet-ct-in-pheochromocytoma-and-paraganglioma-relation-to-genotype-and-amino-acid-transport-system-l
#8
Chloé C Feral, Floriane S Tissot, Lionel Tosello, Nicolas Fakhry, Fréderic Sebag, Karel Pacak, David Taïeb
PURPOSE: F-FDOPA is a highly sensitive and specific radiopharmaceutical for pheochromocytoma and paraganglioma (PPGL) imaging. However, (18)F-FDOPA might be falsely negative in these tumors, especially those related to mutations in succinate dehydrogenase genes (SDHx). The aim of the present study was to evaluate the relationship between expression of L-DOPA transporters and (18)F-FDOPA PET imaging results in PPGL. METHODS: From 2007 to 2015, 175 patients with non-metastatic PPGL were evaluated by (18)F-FDOPA PET/CT for initial diagnosis/staging and follow-up...
November 29, 2016: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/27899258/characterization-of-bioactive-constituents-from-the-gum-resin-of-gardenia-lucida-and-its-pharmacological-potential
#9
Priyanka Maurya, Shilpi Singh, Madan Mohan Gupta, Suaib Luqman
In the present study we mined the information on Gardenia lucida (Dikamali) and identified seven polymethoxyflavones from its gum resin. We also investigated its antiproliferative and antioxidant potential. Xanthomicrol (8) found as potent DPPH scavenger (85.86±1.3%) along with strong ferric plummeting ability (53.60±2.0 FSE) and reducing potential (1.07±0.01) as compared to ascorbic acid. Gardenin B (5) strongly inhibit biochemical production of nitric oxide (IC50 10.59±0.4μg/mL) followed by 5-Desmethylnobiletin (7) and Gardenin E (10, IC5011...
November 26, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27899189/genetic-predisposition-to-kidney-cancer
#10
REVIEW
Laura S Schmidt, W Marston Linehan
Kidney cancer is not a single disease but is made up of a number of different types of cancer classified by histology that are disparate in presentation, clinical course, and genetic basis. Studies of families with inherited renal cell carcinoma (RCC) have provided the basis for our understanding of the causative genes and altered metabolic pathways in renal cancer with different histologies. Von Hippel-Lindau disease was the first renal cancer disorder with a defined genetic basis. Over the next two decades, the genes responsible for a number of other inherited renal cancer syndromes including hereditary papillary renal carcinoma, Birt-Hogg-Dube´syndrome, hereditary leiomyomatosis and renal cell carcinoma, and succinate dehydrogenase-associated renal cancer were identified...
October 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/27898990/european-paediatric-surgeons-association-survey-on-the-management-of-hirschsprung-disease
#11
Augusto Zani, Simon Eaton, Francesco Morini, Prem Puri, Risto Rintala, Ernest van Heurn, Marija Lukac, Pietro Bagolan, Joachim F Kuebler, Florian Friedmacher, Rene Wijnen, Juan A Tovar, Michael E Hoellwarth, Agostino Pierro
: Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61%). DIAGNOSIS: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm...
November 29, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27897402/the-impact-of-ageing-physical-activity-and-pre-frailty-on-skeletal-muscle-phenotype-mitochondrial-content-and-intramyocellular-lipids-in-men
#12
Félix St-Jean-Pelletier, Charlotte H Pion, Jean-Philippe Leduc-Gaudet, Nicolas Sgarioto, Igor Zovilé, Sébastien Barbat-Artigas, Olivier Reynaud, Feras Alkaterji, François C Lemieux, Alexis Grenon, Pierrette Gaudreau, Russell T Hepple, Stéphanie Chevalier, Marc Belanger, José A Morais, Mylène Aubertin-Leheudre, Gilles Gouspillou
BACKGROUND: The exact impact of ageing on skeletal muscle phenotype and mitochondrial and lipid content remains controversial, probably because physical activity, which greatly influences muscle physiology, is rarely accounted for. The present study was therefore designed to investigate the effects of ageing, physical activity, and pre-frailty on skeletal muscle phenotype, and mitochondrial and intramyocellular lipid content in men. METHODS: Recreationally active young adult (20-30 yo; YA); active (ACT) and sedentary (SED) middle-age (50-65 yo; MA-ACT and MA-SED); and older (65 + yo; 65 + ACT and 65 + SED) and pre-frail older (65 + PF) men were recruited...
September 2, 2016: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/27891231/a-pharmacokinetic-evaluation-and-metabolite-identification-of-the-ghb-receptor-antagonist-ncs-382-in-mouse-informs-novel-therapeutic-strategies-for-the-treatment-of-ghb-intoxication
#13
Garrett R Ainslie, K Michael Gibson, Kara R Vogel
Gamma-aminobutyric acid (GABA) is an endogenous inhibitory neurotransmitter and precursor of gamma-hydroxybutyric acid (GHB). NCS-382 (6,7,8,9-tetrahydro-5-hydroxy-5H-benzo-cyclohept-6-ylideneacetic acid), a known GHB receptor antagonist, has shown significant efficacy in a murine model of succinic semialdehyde dehydrogenase deficiency (SSADHD), a heritable neurological disorder featuring chronic elevation of GHB that blocks the final step of GABA degradation. NCS-382 exposures and elimination pathways remain unknown; therefore, the goal of the present work was to obtain in vivo pharmacokinetic data in a murine model and to identify the NCS-382 metabolites formed by mouse and human...
December 2016: Pharmacology Research & Perspectives
https://www.readbyqxmd.com/read/27884696/design-synthesis-and-antifungal-activity-of-novel-fenfuram-diarylamine-hybrids
#14
Hongyu Wang, Xuheng Gao, Xiaoxiao Zhang, Hong Jin, Ke Tao, Taiping Hou
Ten novel fenfuram-diarylamine hybrids were designed and synthesized. And their antifungal activities against four phytopathogenic fungi have been evaluated in vitro and most of the compounds demonstrated a significant antifungal activities against Rhizoctonia solani and Sclerotinia sclerotiorum. Compound 5e exhibited the most potent antifungal activity against R. solani with an EC50 value of 0.037mg/L, far superior to the commercially available fungicide boscalid (EC50=1.71mg/L) and lead fungicide fenfuram (EC50=6...
November 12, 2016: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/27883180/mitochondrial-reactive-oxygen-species-suppress-humoral-immune-response-through-reduction-of-cd19-expression-in-b-cells-in-mice
#15
Masato Ogura, Takeshi Inoue, Junko Yamaki, Miwako K Homma, Tomohiro Kurosaki, Yoshimi Homma
Reactive oxygen species (ROS) are implicated in the modulation of diverse processes including immune responses. To evaluate the effects of metabolic ROS produced by mitochondria on B-cell function and development, we created transgenic (Tg) mice expressing a phosphorylation-defective mutant of succinate dehydrogenase A (SDHA(Y215F) ) in B cells (bSDHA(Y215F) ). Splenic B cells in male, but not female, bSDHA(Y215F) mice produced three times more ROS than those in the control mice, and had decreased production of IgM, IgG1 , and IgG3 , and affinity maturation of IgG1 against T-cell-dependent antigens...
November 24, 2016: European Journal of Immunology
https://www.readbyqxmd.com/read/27875899/amelioration-of-energy-metabolism-by-melatonin-in-skeletal-muscle-of-rats-with-lps-induced-endotoxemia
#16
E Ozkok, H Yorulmaz, G Ates, A Aksu, N Balkis, Ö Şahin, S Tamer
In the literature, few studies have investigated the effects of melatonin on energy metabolism in skeletal muscle in endotoxemia. We investigated the effects of melatonin on tissue structure, energy metabolism in skeletal muscle, and antioxidant level of rats with endotoxemia. We divided rats into 4 groups, control, lipopolysaccharide (LPS) (20 mg/kg, i.p., single dose), melatonin (10 mg/kg, i.p., three times), and melatonin + LPS. Melatonin was injected i.p. 30 min before and after the 2nd and 4th hours of LPS injection...
November 23, 2016: Physiological Research
https://www.readbyqxmd.com/read/27871494/current-surgical-management-of-carotid-body-tumors
#17
Victor J Davila, James M Chang, William M Stone, Richard J Fowl, Thomas C Bower, Michael L Hinni, Samuel R Money
BACKGROUND: Carotid body tumors (CBTs) are rare. Management guidelines may include genetic testing for succinate dehydrogenase (SDH) mutations. We performed an institutional review of the surgical management of CBT. METHODS: A retrospective analysis (1994-2015) of CBT excisions at our institution was performed. Data obtained included demographics, genetic testing (if performed), intraoperative details, postoperative morbidity, and long-term outcomes. Data from the first CBT excision were included in patients with bilateral tumors...
December 2016: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/27868089/adaptive-evolution-of-phosphorus-metabolism-in-prochlorococcus
#18
John R Casey, Adil Mardinoglu, Jens Nielsen, David M Karl
Inorganic phosphorus is scarce in the eastern Mediterranean Sea, where the high-light-adapted ecotype HLI of the marine picocyanobacterium Prochlorococcus marinus thrives. Physiological and regulatory control of phosphorus acquisition and partitioning has been observed in HLI both in culture and in the field; however, the optimization of phosphorus metabolism and associated gains for its phosphorus-limited-growth (PLG) phenotype have not been studied. Here, we reconstructed a genome-scale metabolic network of the HLI axenic strain MED4 (iJC568), consisting of 568 metabolic genes in relation to 794 reactions involving 680 metabolites distributed in 6 subcellular locations...
November 2016: MSystems
https://www.readbyqxmd.com/read/27867439/evaluation-of-head-and-neck-paragangliomas-by-computed-tomography-in-patients-with-pheochromocytoma-paraganglioma-syndromes
#19
Ilona Michałowska, Anna Lewczuk, Jarosław Ćwikła, Aleksander Prejbisz, Urszula Swoboda-Rydz, Mariusz I Furmanek, Małgorzata Szperl, Andrzej Januszewicz, Mariola Pęczkowska
BACKGROUND: Hereditary head and neck paragangliomas (HNP) are very often associated with pheochromocytoma-paraganglioma syndromes, which are caused by mutations in genes encoding subunits of succinate dehydrogenase (SDHx) complex. The aim of this study was to determine the frequency and location of HNP among SDHx carriers. MATERIAL/METHODS: A total of 72 patients with SDHx mutations underwent computed tomography examinations of the head and neck. HNP were present in 44 (61...
2016: Polish Journal of Radiology
https://www.readbyqxmd.com/read/27867097/mitochondrial-remodeling-in-the-liver-following-chronic-alcohol-feeding-to-rats
#20
Derick Han, Heather S Johnson, Madhuri P Rao, Gary Martin, Harsh Sancheti, Kai H Silkwood, Carl W Decker, Kim Tho Nguyen, Joseph G Casian, Enrique Cadenas, Neil Kaplowitz
The feeding of alcohol orally (Lieber-DeCarli diet) to rats has been shown to cause declines in mitochondrial respiration (state III), decreased expression of respiratory complexes, and decreased respiratory control ratios (RCR) in liver mitochondria. These declines and other mitochondrial alterations have led to the hypothesis that alcohol feeding causes "mitochondrial dysfunction" in the liver. If oral alcohol feeding leads to mitochondrial dysfunction, one would predict that increasing alcohol delivery by intragastric (IG) alcohol feeding to rats would cause greater declines in mitochondrial bioenergetics in the liver...
November 17, 2016: Free Radical Biology & Medicine
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