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Succinate dehydrogenase

K R Vogel, G R Ainslie, E E W Jansen, G S Salomons, K M Gibson
Aldehyde dehydrogenase 5a1-deficient (aldh5a1(-/-)) mice, the murine orthologue of human succinic semialdehyde dehydrogenase deficiency (SSADHD), manifest increased GABA (4-aminobutyric acid) that disrupts autophagy, increases mitochondria number, and induces oxidative stress, all mitigated with the mTOR (mechanistic target of rapamycin) inhibitor rapamycin [1]. Because GABA regulates mTOR, we tested the hypothesis that aldh5a1(-/-) mice would show altered levels of mRNA for genes associated with mTOR signaling and oxidative stress that could be mitigated by inhibiting mTOR...
October 16, 2016: Biochimica et Biophysica Acta
Yusuke Matsuda, Noriko Kimura, Takanobu Yoshimoto, Yoshihiro Sekiguchi, Junzo Tomoishi, Ichiro Kasahara, Yoshihito Hara, Yoshihiro Ogawa
Pheochromocytomas and paragangliomas, which exclusively produce dopamine, are very rare. Herein, we report for the first time a Japanese case of an exclusively dopamine-producing paraganglioma accompanied by detailed immunohistochemical analyses. A 70-year-old Japanese woman was referred to our hospital for functional examination of her left retroperitoneal mass. Her adrenal functions were normal, except for excessive dopamine secretion. After the tumorectomy, her dopamine level normalized. The histopathological diagnosis of the tumor was paraganglioma; this was confirmed by positive immunostaining of chromogranin A (CgA), tyrosine hydroxylase (TH), dopamine β-hydroxylase (DBH), and succinate dehydrogenase gene subunit B (SDHB)...
October 14, 2016: Endocrine Pathology
Hsing-Hua Tsai, Shao-Chiang Chang, Cheng-Hsien Chou, Tzu-Pin Weng, Chih-Chin Hsu, Jong-Shyan Wang
This study elucidates how interval and continuous exercise regimens affect the mitochondrial functionality of lymphocytes under hypoxic stress. Sixty healthy sedentary males were randomly assigned to engage in either high-intensity interval training (HIIT, 3 min intervals at 80% and 40% VO2max, n = 20) or moderate-intensity continuous training (MICT, sustained 60% VO2max, n = 20) for 30 min/day, 5 days/week for 6 weeks or were assigned to a control group that did not receive exercise intervention (n = 20)...
October 12, 2016: Scientific Reports
Laura Gálvez, Jéssica Gil-Serna, Marta García, Concepción Iglesias, Daniel Palmero
The most serious aerial disease of garlic is leaf blight caused by Stemphylium spp. Geographical variation in the causal agent of this disease is indicated. Stemphylium vesicarium has been reported in Spain, whereas S. solani is the most prevalent species recorded in China. In this study, Stemphylium isolates were obtained from symptomatic garlic plants sampled from the main Spanish production areas. Sequence data for the ITS1-5.8S-ITS2 region enabled assignation of the isolates to the Pleospora herbarum complex and clearly distinguished the isolates from S...
October 2016: Plant Pathology Journal
Tomoyo Sakata-Kato, Dyann F Wirth
Given that resistance to all drugs in clinical use has arisen, discovery of new anti-malarial drug targets is eagerly anticipated. The Plasmodium mitochondrion has been considered a promising drug target largely based on its significant divergence from the host organelle as well as its involvement in ATP production and pyrimidine biosynthesis. However, the functions of Plasmodium mitochondrial protein complexes and associated metabolic pathways are not fully characterized. Here, we report the development of novel and robust bioenergetic assay protocols for Plasmodium falciparum asexual parasites utilizing a Seahorse Bioscience XFe24 Extracellular Flux Analyzer...
October 9, 2016: ACS Infectious Diseases
Zahra Gerivani, Elham Vashaee, Hamid Reza Sadeghipour, Mahnaz Aghdasi, Zahra-Sadat Shobbar, Majid Azimmohseni
Tree seed dormancy release by cold stratification accompanies with the embryo increased gluconeogenesis competence. Cyanide also breaks seed dormancy however, integrated information about its effects on carbon metabolism is lacking. Accordingly, the impacts of HCN on germination, lipid gluconeogenesis and sugar transport capacity of walnut (Juglans regia L.) kernels were investigated during 10-days period prior to radicle protrusion. HCN increased walnut kernel germination and within four days of kernel incubation, hastened the decline of starch, reducing and non-reducing sugars and led to greater activities of alkaline invertase and glucose-6-phosphate dehydrogenase...
November 2016: Plant Science: An International Journal of Experimental Plant Biology
Xin Zhang, Fei Xie, Baobei Lv, Pengxiang Zhao, Xuemei Ma
A simple and high-throughput assay to detect fungicide resistance is required for large-scale monitoring of the emergence of resistant strains of Botrytis cinerea. Using suspension array technology performed on a Bio-Plex 200 System, we developed a single-tube allele-specific primer extension assay that can simultaneously detect eight alleles in one reaction. These eight alleles include E198 and 198A of the β-Tubulin gene (BenA), H272 and 272Y of the Succinate dehydrogenase iron-sulfur subunit gene (SdhB), I365 and 365S of the putative osmosensor histidine kinase gene (BcOS1), and F412 and 412S of the 3-ketoreductase gene (erg27)...
2016: Frontiers in Microbiology
Xinyan Ma, Xiudong Liao, Lin Lu, Sufen Li, Liyang Zhang, Xugang Luo
BACKGROUND: The current dietary iron requirement (80 mg/kg) of broilers is mainly based on growth, hemoglobin concentration, or hematocrit data obtained in a few early studies; however, expressions of iron-containing enzymes might be more sensitive novel criteria to evaluate dietary iron requirements. OBJECTIVE: The objective of this study was to determine dietary iron requirements of broilers for the full expression of succinate dehydrogenase (SDH), catalase, and cytochrome c oxidase (COX) in various tissues...
October 5, 2016: Journal of Nutrition
Sarah H White, Mary M McDermott, Robert L Sufit, Kate Kosmac, Alex W Bugg, Marta Gonzalez-Freire, Luigi Ferrucci, Lu Tian, Lihui Zhao, Ying Gao, Melina R Kibbe, Michael H Criqui, Christiaan Leeuwenburgh, Charlotte A Peterson
BACKGROUND: Patients with lower extremity peripheral artery disease (PAD) have decreased mobility, which is not fully explained by impaired blood supply to the lower limb. Additionally, reports are conflicted regarding fiber type distribution patterns in PAD, but agree that skeletal muscle mitochondrial respiration is impaired. METHODS: To test the hypothesis that reduced muscle fiber oxidative activity and type I distribution are negatively associated with walking performance in PAD, calf muscle biopsies from non-PAD (n = 7) and PAD participants (n = 26) were analyzed immunohistochemically for fiber type and size, oxidative activity, markers of autophagy, and capillary density...
September 29, 2016: Journal of Translational Medicine
E E Jansen, K R Vogel, G S Salomons, P L Pearl, J-B Roullet, K M Gibson
We hypothesized that blood levels of γ-aminobutyric acid (GABA) and γ-hydroxybutyric acid (GHB), biomarkers of succinic semialdehyde dehydrogenase deficiency (SSADHD), would correlate with age. GABA and GHB were quantified in plasma and red blood cells (RBCs) from 18 patients (age range 5-41 years; median 8). Both metabolites negatively correlated with age (P < 0.05). Plasma and RBC GHB declined with age, reaching a nadir and approximate steady state by 10 years. Declining plasma GABA achieved this approximate steady state at 30-40 years of age...
November 2016: Journal of Inherited Metabolic Disease
Aaron M Udager, Rohit Mehra
Molecular and morphologic interrogation has driven a much-needed reexamination of renal cell carcinoma (RCC). Indeed, the recently released 2016 World Health Organization classification now recognizes 12 distinct RCC subtypes, as well as several other emerging/provisional RCC entities. From a clinical perspective, accurate RCC classification may have important implications for patients and their families, including prognostic risk stratification, targeted therapeutics selection, and identification for genetic testing...
October 2016: Archives of Pathology & Laboratory Medicine
Carolina Courage, Christopher B Jackson, Dagmar Hahn, Liliya Euro, Jean-Marc Nuoffer, Sabina Gallati, André Schaller
Isolated defects of the mitochondrial respiratory complex II (succinate dehydrogenase, SDH) are rare, accounting for approximately 2% of all respiratory chain deficiency diagnoses. Here, we report clinical and molecular investigations of three family members with a heterozygous mutation in the large flavoprotein subunit SDHA previously described to cause complex II deficiency. The index patient presented with bilateral optic atrophy and ocular movement disorder, a progressive polyneuropathy, psychiatric involvement, and cardiomyopathy...
September 28, 2016: American Journal of Medical Genetics. Part A
Nicola Tufton, Lucy Shapiro, Umasuthan Srirangalingam, Polly Richards, Anju Sahdev, V K Ajith Kumar, Lorraine McAndrew, Lee Martin, Daniel Berney, John Monson, Shern L Chew, Mona Waterhouse, Maralyn Druce, Márta Korbonits, Karl Metcalfe, William M Drake, Helen L Storr, Scott A Akker
OBJECTIVE: For 'asymptomatic carriers' of the succinate dehydrogenase subunit B (SDHB) gene mutations, there is currently no consensus as to the appropriate modality or frequency of surveillance imaging. We present the results of a surveillance programme of SDHB mutation carriers. DESIGN: Review of clinical outcomes of a surveillance regimen in patients identified to have a SDHB gene mutation, based on annual MRI, in a single UK tertiary referral centre. PATIENTS: A total of 92 patients were identified with an SDHB gene mutation...
September 28, 2016: Clinical Endocrinology
Xiaoxiang Dong, Xiulai Chen, Yuanyuan Qian, Yuancai Wang, Li Wang, Weihua Qiao, Liming Liu
A four-carbon dicarboxylic acid L-malate has recently attracted attention due to its potential applications in the fields of medicine and agriculture. In this study, Escherichia coli W3110 was engineered and optimized for L-malate production via one-step L-malate synthesis pathway. First, deletion of the genes encoding lactate dehydrogenase (ldhA), pyruvate oxidase (poxB), pyruvate formate lyase (pflB), phosphotransacetylase (pta), and acetate kinase A (ackA) in pta-ackA pathway led to accumulate 20.9 g/L pyruvate...
September 26, 2016: Biotechnology and Bioengineering
Evanna L Mills, Beth Kelly, Angela Logan, Ana S H Costa, Mukund Varma, Clare E Bryant, Panagiotis Tourlomousis, J Henry M Däbritz, Eyal Gottlieb, Isabel Latorre, Sinéad C Corr, Gavin McManus, Dylan Ryan, Howard T Jacobs, Marten Szibor, Ramnik J Xavier, Thomas Braun, Christian Frezza, Michael P Murphy, Luke A O'Neill
Activated macrophages undergo metabolic reprogramming, which drives their pro-inflammatory phenotype, but the mechanistic basis for this remains obscure. Here, we demonstrate that upon lipopolysaccharide (LPS) stimulation, macrophages shift from producing ATP by oxidative phosphorylation to glycolysis while also increasing succinate levels. We show that increased mitochondrial oxidation of succinate via succinate dehydrogenase (SDH) and an elevation of mitochondrial membrane potential combine to drive mitochondrial reactive oxygen species (ROS) production...
October 6, 2016: Cell
Luis V Portela, Andressa W Brochier, Clarissa B Haas, Afonso Kopczynski de Carvalho, Jussania A Gnoato, Eduardo R Zimmer, Eduardo Kalinine, Luc Pellerin, Alexandre P Muller
Hyperpalatable diets (HP) impair brain metabolism, and regular physical exercise has an apparent opposite effect. Here, we combined a prior long-term exposure to HP diet followed by physical exercise and evaluated the impact on some neuroenergetic components and on cognitive performance. We assessed the extracellular lactate concentration, expression of monocarboxylate transporters (MCTs), pyruvate dehydrogenase (PDH), and mitochondrial function in the hippocampus. Male C57BL/6J mice were fed 4 months with HP or a control diet...
September 22, 2016: Molecular Neurobiology
Marta J Piotrowska, James M Fountaine, Richard A Ennos, Maciej Kaczmarek, Fiona J Burnett
BACKGROUND: Ramularia collo-cygni (Rcc) is responsible for Ramularia leaf spot (RLS), a foliar disease of barley contributing to serious economic losses. Protection against the disease has been almost exclusively based on fungicide applications, including Succinate Dehydrogenase Inhibitors (SDHIs). In 2015 the first field isolates of Rcc with reduced sensitivity to SDHIs were recorded in some European countries. In this study we established baseline sensitivity of Rcc to SDHIs in the UK and characterised mutations correlating with resistance to SDHIs in UV-generated mutants...
September 19, 2016: Pest Management Science
Eleni Daniel, Robert Jones, Matthew Bull, John Newell-Price
INTRODUCTION: Patients with SDHx mutations need long-term radiological surveillance for the development of paragangliomas and phaeochromocytomas, but no longitudinal data exist. We assessed the performance of rapid-sequence non-contrast magnetic resonance imaging (MRI) in the long-term monitoring of patients with SDHx mutations. METHODS: Retrospective study between 2005-2015 at a University Hospital and regional endocrine genetics referral center. Clinical and imaging data of forty-seven patients with SDHx mutations [SDHB (36), SDHC (6), SDHD (5)] who had surveillance for detection of paragangliomas by rapid-sequence non-contrast MRI (base of skull to pubic symphysis) were collected...
September 15, 2016: European Journal of Endocrinology
Michael F A Bradfield, Willie Nicol
Increased pentose phosphate pathway flux, relative to total substrate uptake flux, is shown to enhance succinic acid (SA) yields under continuous, non-growth conditions of Actinobacillus succinogenes biofilms. Separate fermentations of glucose and xylose were conducted in a custom, continuous biofilm reactor at four different dilution rates. Glucose-6-phosphate dehydrogenase assays were performed on cell extracts derived from in situ removal of biofilm at each steady state. The results of the assays were coupled to a kinetic model that revealed an increase in oxidative pentose phosphate pathway (OPPP) flux relative to total substrate flux with increasing SA titre, for both substrates...
September 9, 2016: Applied Microbiology and Biotechnology
N T Gaisa
The article describes the tumorigenesis of bladder cancer from a pathological perspective in three dimensions: morphology, genetics and epigenetics. Field cancerization and tumor cell migration/seeding are the two main hypotheses used for explaining synchronous and metachronous tumors in the urinary tract. By detailed histological mapping of completely embedded cystectomy specimens we found a single tumor focus in nearly 2/3 of the bladders accompanied by surrounding preinvasive carcinoma in situ. We substantiated our findings by studies analyzing TP53 mutations and loss of heterozygosity in various tumor sites...
September 9, 2016: Der Pathologe
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