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Succinate dehydrogenase

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https://www.readbyqxmd.com/read/28796048/clinicopathologic-study-of-succinate-dehydrogenase-deficient-gastrointestinal-stromal-tumors-a-single-institutional-experience-in-china
#1
Weizhen Liu, Xiangyu Zeng, Xiuli Wu, Jun He, Jinbo Gao, Xiaoming Shuai, Guobin Wang, Peng Zhang, Kaixiong Tao
Gastrointestinal stromal tumors (GISTs) that are not driven by kinase mutations, as are most GISTs, often show loss of function of the succinate dehydrogenase (SDH) complex and are considered SDH-deficient GISTs. SDH-deficient GISTs share many distinct characteristics compared with conventional GISTs. However, data regarding these characteristics, particularly among Asian people, are relatively limited. The objective of this study was to characterize the clinicopathologic characteristics, treatment, and prognosis of these uncommon GISTs...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28795281/selective-a2a-receptor-antagonist-sch-58261-modulates-striatal-oxidative-stress-and-alleviates-toxicity-induced-by-3-nitropropionic-acid-in-male-wistar-rats
#2
Cristiani F Bortolatto, Angélica S Reis, Mikaela P Pinz, Guilherme T Voss, Renata L Oliveira, Ane G Vogt, Silvane Roman, Cristiano R Jesse, Cristiane Luchese, Ethel A Wilhelm
The aim of the present study was to investigate the effects of SCH58261, a selective adenosine A2A receptor antagonist, on striatal toxicity induced by 3-nitropropionic acid (3-NP) in rats. The experimental protocol consisted of 10 administrations (once a day) of SCH58261 (0.01 or 0.05 mg/kg/day, intraperitoneal, i.p.). From 7th to 10th day, 3-NP (20 mg/kg/day, i.p.) was injected 1 h after SCH58261 administration. Twenty-four hours after the last 3-NP injection, the body weight gain, locomotor activity (open-field test), motor coordination (rotarod test), striatal succinate dehydrogenase (SDH) activity and parameters linked to striatal oxidative status were evaluated in rats...
August 9, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28794154/both-brown-adipose-tissue-and-skeletal-muscle-thermogenesis-processes-are-activated-during-mild-to-severe-cold-adaptation-in-mice
#3
Naresh C Bal, Sushant Singh, Felipe C G Reis, Santosh K Maurya, Sunil Pani, Leslie A Rowland, Muthu Periasamy
Thermogenesis is an important homeostatic mechanism, essential for survival and normal physiological functions in mammals. Both brown adipose tissue (BAT) (i.e. uncoupling protein 1 [UCP1]-based) and skeletal muscle (i.e. sarcolipin [SLN]-based) thermogenesis processes play important roles in temperature homeostasis, but their relative contributions differ from small to large mammals. In this study, we investigated the functional interplay between skeletal muscle and BAT-based thermogenesis under mild versus severe cold adaptation employing UCP1-/- and SLN-/- mice...
August 9, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28781258/-metabolic-changes-in-pulmonary-mitochondria-of-rats-with-experimental-hyperhomocysteinemia
#4
D V Medvedev, V I Zvyagina, O M Uryasev, E S Belskikh, S V Bulatetskiy, A N Ryabkov
Hyperhomocysteinemia is a risk factor for many human diseases, including pulmonary pathologies. In this context much interest attracts secondary mitochondrial dysfunction, which is an important link in pathogenesis of diseases associated with hyperhomocysteinemia. The study was conducted using male Wistar rats. It was found that under conditions of severe hyperhomocysteinemia caused by administration of methionine, homocysteine was accumulated in lung mitochondria thus suggesting a direct toxic effect on these organelles...
May 2017: Biomedit︠s︡inskai︠a︡ Khimii︠a︡
https://www.readbyqxmd.com/read/28769919/cellular-response-to-ciprofloxacin-in-low-level-quinolone-resistant-escherichia-coli
#5
Jesús Machuca, Esther Recacha, Alejandra Briales, Paula Díaz-de-Alba, Jesús Blazquez, Álvaro Pascual, José-Manuel Rodríguez-Martínez
Bactericidal activity of quinolones has been related to a combination of DNA fragmentation, reactive oxygen species (ROS) production and programmed cell death (PCD) systems. The underlying molecular systems responsible for reducing bactericidal effect during antimicrobial therapy in low-level quinolone resistance (LLQR) phenotypes need to be clarified. To do this and also define possible new antimicrobial targets, the transcriptome profile of isogenic Escherichia coli harboring quinolone resistance mechanisms in the presence of a clinical relevant concentration of ciprofloxacin was evaluated...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28768491/succinate-dehydrogenase-deficiency-in-a-pdgfra-mutated-gist
#6
Martin G Belinsky, Kathy Q Cai, Yan Zhou, Biao Luo, Jianming Pei, Lori Rink, Margaret von Mehren
BACKGROUND: Most gastrointestinal stromal tumors (GISTs) harbor mutually exclusive gain of function mutations in the receptor tyrosine kinase (RTK) KIT (70-80%) or in the related receptor PDGFRA (~10%). These GISTs generally respond well to therapy with the RTK inhibitor imatinib mesylate (IM), although initial response is genotype-dependent. An alternate mechanism leading to GIST oncogenesis is deficiency in the succinate dehydrogenase (SDH) enzyme complex resulting from genetic or epigenetic inactivation of one of the four SDH subunit genes (SDHA, SDHB, SDHC, SDHD, collectively referred to as SDHX)...
August 2, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28764992/impact-on-gaba-systems-in-monogenetic-developmental-cns-disorders-clues-to-symptomatic-treatment
#7
REVIEW
Dietmar Benke, Hanns Möhler
Animal studies of several single-gene disorders demonstrate that reversing the molecular signaling deficits can result in substantial symptomatic improvements in function. Focusing on the ratio of excitation to inhibition as a potential pathophysiological hallmark, seven single-gene developmental CNS disorders are reviewed which are characterized by a striking dysregulation of neuronal inhibition. Deficits in inhibition and excessive inhibition are found. The examples of developmental disorders encompass Neurofibromatosis type 1, Fragile X syndrome, Rett syndrome, Dravet syndrome including autism-like behavior, NONO-mutation-induced intellectual disability, Succinic semialdehyde dehydrogenase deficiency and Congenital nystagmus due to FRMD7 mutations...
July 29, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28763556/modifications-in-retinal-mitochondrial-respiration-precede-type-2-diabetes-and-protracted-microvascular-retinopathy
#8
Woo Hyun Han, Jonathan Gotzmann, Sharee Kuny, Hui Huang, Catherine B Chan, Hélène Lemieux, Yves Sauvé
Purpose: To characterize retinal mitochondrial respiration associated with type 2 diabetes (T2D) progression in a cone-rich diurnal rodent, the Nile rat (genus Arvicanthis, species niloticus). Methods: Nile rats were fed a standard rodent diet that resulted in rising glucose levels from 6 months. Age-matched control animals were fed a high-fiber diet that prevented diabetes up to 18 months. The functional status of specific retinal mitochondrial components and mitochondrial outer membrane integrity were studied by using high-resolution respirometry...
August 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28752484/composite-pheochromocytoma-paraganglioma-ganglioneuroma-a-clinicopathologic-study-of-eight-cases-with-analysis-of-succinate-dehydrogenase
#9
Sounak Gupta, Jun Zhang, Lori A Erickson
Ganglioneuromas represent the most well-differentiated spectrum of neoplasia arising from the sympathetic nervous system, while neuroblastomas represent the most poorly differentiated counterpart, and ganglioneuroblastomas represent intermediate stages of differentiation. Small series of cases have documented the co-occurrence of ganglioneuroma with a pheochromocytoma (Pheo)/paraganglioma (PGL) component. We report the clinicopathologic features of eight such cases, diagnosed between 2003 and 2015 with a mean follow-up of 22 months (1-47), which were evaluated for syndrome associations, SDHB expression, and clinical outcome...
July 27, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28749584/the-aldh21-gene-found-in-lower-plants-and-some-vascular-plants-codes-for-a-nadp-dependent-succinic-semialdehyde-dehydrogenase
#10
Martina Kopečná, Armelle Vigouroux, Jan Vilím, Radka Končitíková, Pierre Briozzo, Eva Hájková, Lenka Jašková, Klaus von Schwartzenberg, Marek Šebela, Solange Moréra, David Kopečný
Lower plant species including some green algae, non-vascular plants (bryophytes) as well as the oldest vascular plants (lycopods) and ferns (monilophytes) possess a unique aldehyde dehydrogenase (ALDH) gene named ALDH21, which is upregulated during dehydration. However, the gene is absent in flowering plants. Here, we show that ALDH21 from the moss Physcomitrella patens codes for a tetrameric NADP(+) -dependent succinic semialdehyde dehydrogenase (SSALDH), which converts succinic semialdehyde, an intermediate of the γ-aminobutyrate (GABA) shunt pathway, into succinate in the cytosol...
July 27, 2017: Plant Journal: for Cell and Molecular Biology
https://www.readbyqxmd.com/read/28743823/unloading-induced-atrophy-and-decreased-oxidative-capacity-of-the-soleus-muscle-in-rats-are-reversed-by-pre-and-postconditioning-with-mild-hyperbaric-oxygen
#11
Ai Takemura, Roland R Roy, Ikumi Yoshihara, Akihiko Ishihara
Our aim was to determine the effects of pre- and/or postconditioning with mild hyperbaric oxygen (1.25 atmospheric pressure, 36% oxygen for 3 h/day) on the properties of the soleus muscle that was atrophied by hindlimb suspension-induced unloading. Twelve groups of 8-week-old rats were housed under normobaric conditions (1 atmospheric pressure, 20.9% oxygen) or exposed to mild hyperbaric oxygen for 2 weeks. Ten groups then were housed under normobaric conditions for 2 weeks with their hindlimbs either unloaded via suspension or not unloaded...
July 2017: Physiological Reports
https://www.readbyqxmd.com/read/28739378/succinate-dehydrogenase-sdh-deficiency-carney-triad-and-the-epigenome
#12
REVIEW
Nikolaos Settas, Fabio R Faucz, Constantine A Stratakis
In this report, we review the relationship between succinate dehydrogenase (SDH) deficiency and the epigenome, especially with regards to two clinical conditions. Carney triad (CT) is a very rare disease with synchronous or metachronous occurrence of at least three different tumor entities; gastric gastrointestinal stromal tumor (GIST), paraganglioma (PGL), and pulmonary chondroma. This condition affects mostly females and it is never inherited. Another disease that shares two of the tumor components of CT, namely GIST and PGL is the Carney-Stratakis syndrome (CSS) or dyad...
July 21, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28738844/analysis-of-sdhaf3-in-familial-and-sporadic-pheochromocytoma-and-paraganglioma
#13
Trisha Dwight, Un Na, Edward Kim, Ying Zhu, Anne Louise Richardson, Bruce G Robinson, Katherine M Tucker, Anthony J Gill, Diana E Benn, Roderick J Clifton-Bligh, Dennis R Winge
BACKGROUND: Germline mutations in genes encoding subunits of succinate dehydrogenase (SDH) are associated with the development of pheochromocytoma (PC) and/or paraganglioma (PGL). As assembly factors have been identified as playing a role in maturation of individual SDH subunits and assembly of the functioning SDH complex, we hypothesized that SDHAF3 variants may be associated with PC/PGL and functionality of SDH. METHODS: DNA was extracted from the blood of 37 individuals (from 23 families) with germline SDH mutations and 18 PC/PGL (15 sporadic, 3 familial) and screened for mutations using a custom gene panel, containing SDHAF3 (SDH assembly factor 3) as well as eight known PC/PGL susceptibility genes...
July 24, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28736181/succinate-accumulation-impairs-cardiac-pyruvate-dehydrogenase-activity-through-grp91-dependent-and-independent-signaling-pathways-therapeutic-effects-of-ginsenoside-rb1
#14
Jia Li, Yi-Lin Yang, Lan-Zhu Li, Lei Zhang, Qun Liu, Kang Liu, Ping Li, Baolin Liu, Lian-Wen Qi
Altered mitochondrial oxidation increases vulnerability to cardiac ischemia/reperfusion (I/R) injury in metabolic disorders. However, the metabolic signaling responsible for the dysfunction remains partly unknown. We sought to test whether or not hypoxic succinate accumulation could inhibit pyruvate dehydrogenase (PDH) activity and subsequently aggravate I/R injury. Results showed that saturated fatty acid palmitate stimulation increased fatty acid oxidation and induced hypoxia in cardiomyocytes, leading to succinate accumulation...
July 20, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28730528/sulforaphane-promotes-mitochondrial-protection-in-sh-sy5y-cells-exposed-to-hydrogen-peroxide-by-an-nrf2-dependent-mechanism
#15
Marcos Roberto de Oliveira, Flávia de Bittencourt Brasil, Cristina Ribas Fürstenau
Sulforaphane (SFN; C6H11NOS2) is an isothiocyanate found in cruciferous vegetables, such as broccoli, kale, and radish. SFN exhibits antioxidant, anti-apoptotic, anti-tumor, and anti-inflammatory activities in different cell types. However, it was not previously demonstrated whether and how this natural compound would exert mitochondrial protection experimentally. Therefore, we investigated here the effects of a pretreatment (for 30 min) with SFN at 5 μM on mitochondria obtained from human neuroblastoma SH-SY5Y cells exposed to hydrogen peroxide (H2O2) at 300 μM for 24 h...
July 20, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28725638/isolation-of-f-novicida-containing-phagosome-from-infected-human-monocyte-derived-macrophages
#16
Valentina Marecic, Olga Shevchuk, Mateja Ozanic, Mirna Mihelcic, Michael Steinert, Antonija Jurak Begonja, Yousef Abu Kwaik, Marina Santic
Francisella is a gram-negative bacterial pathogen, which causes tularemia in humans and animals. A crucial step of Francisella infection is its invasion of macrophage cells. Biogenesis of the Francisella-containing phagosome (FCP) is arrested for ~15 min at the endosomal stage, followed by gradual bacterial escape into the cytosol, where the microbe proliferates. The crucial step in pathogenesis of tularemia is short and transient presence of the bacterium within phagosome. Isolation of FCPs for further studies has been challenging due to the short period of time of bacterial residence in it and the characteristics of the FCP...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28724664/biochemical-molecular-and-clinical-characterization-of-succinate-dehydrogenase-subunit-a-variants-of-unknown-significance
#17
Amber E Bannon, Jason D Kent, Isaac Forquer, Ajia Town, Lillian R Klug, Kelly E McCann, Carol Beadling, Olivier Harismendy, Jason K Sicklick, Christopher L Corless, Ujwal Shinde, Michael C Heinrich
Patients who inherit a pathogenic loss-of-function genetic variant involving one of the four succinate dehydrogenase (SDH) subunit genes have up to an 86% chance of developing one or more cancers by the age of 50. If tumors are identified and removed early in these high-risk patients, they have a higher potential for cure.  Unfortunately, many alterations identified in these genes are variants of unknown significance (VUS), confounding the identification of high-risk patients. If we could identify misclassified SDH VUS as benign or pathogenic SDH mutations, we could better select patients for cancer screening procedures and remove tumors at earlier stages...
July 19, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28718507/gene-expression-analyses-reveal-metabolic-specifications-in-acute-o2-sensing-chemoreceptor-cells
#18
Lin Gao, Victoria Bonilla-Henao, Paula García-Flores, Ignacio Arias-Mayenco, Patricia Ortega-Sáenz, José López-Barneo
Acute O2 sensing is a fundamental property of cells in the peripheral chemoreceptors, e.g. glomus cells in the carotid body (CB) and chromaffin cells in the adrenal medulla (AM), and is necessary for adaptation to hypoxia. These cells contain O2 -sensitive ion channels, which mediate membrane depolarization and transmitter release upon exposure to hypoxia. However, the mechanisms underlying the detection of changes in O2 tension by cells are still poorly understood. Recently, we suggested that CB glomus cells have specific metabolic features that favour the accumulation of reduced quinone and the production of mitochondrial NADH and reactive oxygen species during hypoxia...
July 17, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28716098/awakening-sleeping-beauty-production-of-propionic-acid-in-escherichia-coli-through-the-sbm-operon-requires-the-activity-of-a-methylmalonyl-coa-epimerase
#19
Ricardo Axayacatl Gonzalez-Garcia, Tim McCubbin, Annalena Wille, Manuel Plan, Lars Keld Nielsen, Esteban Marcellin
BACKGROUND: Propionic acid is used primarily as a food preservative with smaller applications as a chemical building block for the production of many products including fabrics, cosmetics, drugs, and plastics. Biological production using propionibacteria would be competitive against chemical production through hydrocarboxylation of ethylene if native producers could be engineered to reach near-theoretical yield and good productivity. Unfortunately, engineering propionibacteria has proven very challenging...
July 17, 2017: Microbial Cell Factories
https://www.readbyqxmd.com/read/28710913/expression-and-promoter-methylation-of-succinate-dehydrogenase-and-fumarase-genes-in-maize-under-anoxic-conditions
#20
Alexander T Eprintsev, Dmitry N Fedorin, Maria A Dobychina, Abir U Igamberdiev
Succinate dehydrogenase (SDH) and fumarase enzyme activity and expression of genes encoding the SDH subunits A (Sdh1-2), B (Sdh2-3), C (Sdh3), D (Sdh4) and the mitochondrial (Fum1) and cytosolic (Fum2) isoforms of fumarase were quantified in maize (Zea mays L.) seedlings exposed to atmospheres of air (control), N2, and CO2. The catalytic activity of SDH gradually declined in plants exposed to N2 atmospheres, with ∼40% activity remaining after 24h. In seedlings incubated in CO2, the suppression was even more pronounced...
September 2017: Journal of Plant Physiology
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