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Mitochondrial creatine kinase

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https://www.readbyqxmd.com/read/28812460/myopathic-mtdna-depletion-syndrome-due-to-mutation-in-tk2-gene
#1
Elena Martín-Hernández, María Teresa García-Silva, Pilar Quijada-Fraile, María Elena Rodríguez-García, Henry Rivera, Aurelio Hernández-Laín, David Coca-Robinot, Joaquín Fernández-Toral, Joaquín Arenas, Miguel A Martín, Francisco Martínez-Azorín
Whole-exome sequencing was used to identify the disease gene(s) in a Spanish girl with failure to thrive, muscle weakness, mild facial weakness, elevated creatine kinase, deficiency of mitochondrial complex III and depletion of mtDNA. With whole-exome sequencing data, it was possible to get the whole mtDNA sequencing and discard any pathogenic variant in this genome. The analysis of whole exome uncovered a homozygous pathogenic mutation in thymidine kinase 2 gene ( TK2; NM_004614.4:c.323 C>T, p.T108M)...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28807772/streptococcus-agalactiae-impairs-cerebral-bioenergetics-in-experimentally-infected-silver-catfish
#2
Matheus D Baldissera, Carine F Souza, Belisa S Parmeggiani, Roberto C V Santos, Guilhian Leipnitz, Karen L S Moreira, Maria Izabel U M da Rocha, Marcelo L da Veiga, Bernardo Baldisserotto
It is becoming evident that bacterial infectious diseases affect brain energy metabolism, where alterations of enzymatic complexes of the mitochondrial respiratory chain and creatine kinase (CK) lead to an impairment of cerebral bioenergetics which contribute to disease pathogenesis in the central nervous system (CNS). Based on this evidence, the aim of this study was to evaluate whether alterations in the activity of complex IV of the respiratory chain and CK contribute to impairment of cerebral bioenergetics during Streptococcus agalactiae infection in silver catfish (Rhamdia quelen)...
August 11, 2017: Microbial Pathogenesis
https://www.readbyqxmd.com/read/28806770/increasing-creatine-kinase-activity-protects-against-hypoxia-reoxygenation-injury-but-not-against-anthracycline-toxicity-in-vitro
#3
Sevasti Zervou, Hannah J Whittington, Philip J Ostrowski, Fang Cao, Jack Tyler, Hannah A Lake, Stefan Neubauer, Craig A Lygate
The creatine kinase (CK) phosphagen system is fundamental to cellular energy homeostasis. Cardiomyocytes express three CK isoforms, namely the mitochondrial sarcomeric CKMT2 and the cytoplasmic CKM and CKB. We hypothesized that augmenting CK in vitro would preserve cell viability and function and sought to determine efficacy of the various isoforms. The open reading frame of each isoform was cloned into pcDNA3.1, followed by transfection and stable selection in human embryonic kidney cells (HEK293). CKMT2- CKM- and CKB-HEK293 cells had increased protein and total CK activity compared to non-transfected cells...
2017: PloS One
https://www.readbyqxmd.com/read/28792616/proteomic-profiling-of-liver-and-plasma-in-chronic-ethanol-feeding-model-of-hepatic-alcohol-dehydrogenase-deficient-deer-mice
#4
Kamlesh K Bhopale, Samir M Amer, Lata Kaphalia, Kizhake V Soman, John E Wiktorowicz, G A Shakeel Ansari, Bhupendra S Kaphalia
BACKGROUND: Chronic alcohol abuse, a major risk factor for such diseases as hepatitis and cirrhosis, impairs hepatic alcohol dehydrogenase (ADH, key ethanol metabolizing enzyme). Therefore, differentially altered hepatic and plasma proteomes were identified in chronic ethanol feeding model of hepatic ADH-deficient (ADH(-) ) deer mice to understand the metabolic basis of alcoholic liver disease (ALD). METHODS: ADH(-) deer mice were fed 3.5g% ethanol via Lieber-DeCarli liquid diet daily for three months, and histology of the liver assessed...
August 9, 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/28772214/epigallocatechin-3-gallate-protects-against-diabetic-cardiomyopathy-through-modulating-the-cardiometabolic-risk-factors-oxidative-stress-inflammation-cell-death-and-fibrosis-in-streptozotocin-nicotinamide-induced-diabetic-rats
#5
Azza I Othman, Mamdouh R El-Sawi, Mohamed A El-Missiry, Mohammad H Abukhalil
The potential protective effect of epigallocatechin-3-gallate (EGCG) on type 2 diabetes-induced heart injury was investigated. A rat model of diabetes was achieved by injection of nicotinamide (100mg/kg, i.p), 20min before the administration of streptozotocin (55mg/kg, i.p.). After confirmation of diabetes, EGCG (2mg/kg, p.o.) was administrated on alternate days for one month. Treatment of diabetic rats with EGCG showed a remarkable reduction in glucose, glycosylated hemoglobin, HOMA-IR and lipid profile levels with an elevation in insulin levels, indicating its antihyperglycemic and antidyslipidemic actions...
July 31, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28745369/chlorogenic-acid-protects-against-aluminium-induced-cytotoxicity-through-chelation-and-antioxidant-actions-in-primary-hippocampal-neuronal-cells
#6
Xiaomei Wang, Xinguang Fan, Shuzhi Yuan, Wenxiao Jiao, Bangdi Liu, Jiankang Cao, Weibo Jiang
Chlorogenic acid (CGA), a major polyphenolic component of many plants, displays antioxidant and neuroprotective properties in neurodegenerative diseases. To investigate whether CGA may influence aluminium (Al) induced cytotoxicity, aluminium chloride (50 μM Al) was administered in primary hippocampal neuronal cells presupplemented with CGA (10, 50 and 100 μM). Our study shows that the exposure to Al caused cell death, Al(3+) accumulation, reactive oxygen species generation and mitochondrial damage in cells...
August 1, 2017: Food & Function
https://www.readbyqxmd.com/read/28737531/potential-causes-of-elevated-ree-following-high-intensity-exercise
#7
Gary R Hunter, Douglas R Moellering, Stephen J Carter, Barbara A Gower, Marcas M Bamman, Lyndsey M Hornbuckle, Eric P Plaisance, Gordon Fisher
INTRODUCTION: Resting energy expenditure (REE) increases following intense exercise; however, little is known concerning mechanisms. PURPOSE: Determine effects of a single bout of moderate-intensity continuous aerobic exercise (MIC), or high intensity interval exercise (HII) on REE under energy balance conditions. METHODS: Thirty-three untrained premenopausal women were evaluated at baseline, after 8-16 weeks of training, 22 hours following either MIC (50% peak VO2) or HII (84% peak VO2)...
July 21, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/28735082/aeromonas-caviae-alters-the-cytosolic-and-mitochondrial-creatine-kinase-activities-in-experimentally-infected-silver-catfish-impairment-on-renal-bioenergetics
#8
Matheus D Baldissera, Carine F Souza, Guerino B Júnior, Camila Marina Verdi, Karen L S Moreira, Maria Izabel U M da Rocha, Marcelo L da Veiga, Roberto C V Santos, Bruno S Vizzotto, Bernardo Baldisserotto
Cytosolic and mitochondrial creatine kinases (CK), through the creatine kinase-phosphocreatine (CK/PCr) system, provide a temporal and spatial energy buffer to maintain cellular energy homeostasis. However, the effects of bacterial infections on the kidney remain poorly understood and are limited only to histopathological analyses. Thus, the aim of this study was to investigate the involvement of cytosolic and mitochondrial CK activities in renal energetic homeostasis in silver catfish experimentally infected with Aeromonas caviae...
July 19, 2017: Microbial Pathogenesis
https://www.readbyqxmd.com/read/28624490/bioenergetics-dysfunction-mitochondrial-permeability-transition-pore-opening-and-lipid-peroxidation-induced-by-hydrogen-sulfide-as-relevant-pathomechanisms-underlying-the-neurological-dysfunction-characteristic-of-ethylmalonic-encephalopathy
#9
Gabriela Miranda Fernandez Cardoso, Julia Tauana Pletsch, Belisa Parmeggiani, Mateus Grings, Nícolas Manzke Glanzel, Larissa Daniele Bobermin, Alexandre Umpierrez Amaral, Moacir Wajner, Guilhian Leipnitz
Hydrogen sulfide (sulfide) accumulates at high levels in brain of patients with ethylmalonic encephalopathy (EE). In the present study, we evaluated whether sulfide could disturb energy and redox homeostasis, and induce mitochondrial permeability transition (mPT) pore opening in rat brain aiming to better clarify the neuropathophysiology of EE. Sulfide decreased the activities of citrate synthase and aconitase in rat cerebral cortex mitochondria, and of creatine kinase (CK) in rat cerebral cortex, striatum and hippocampus supernatants...
June 15, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28612295/neuroprotective-effect-of-creatine-and-pyruvate-on-enzyme-activities-of-phosphoryl-transfer-network-and-oxidative-stress-alterations-caused-by-leucine-administration-in-wistar-rats
#10
Elenara Rieger, Itiane Diehl de Franceschi, Thales Preissler, Clovis Milton Duval Wannmacher
Maple syrup urine disease is an autosomal metabolic disease caused by a deficiency of branched-chain α-keto acid dehydrogenase complex activity. In this disease occur the accumulation of the branched-chain amino acids leucine, isoleucine, and valine and their corresponding branched-chain α-keto acids in the tissues and body fluids. The affected patients may present psychomotor development delay and mental retardation. The pathophysiology of maple syrup urine disease is not entirely understood, but leucine seems to be the primary neurotoxic metabolite...
June 13, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28605332/protective-effect-of-curcumin-against-carbofuran-induced-toxicity-in-wistar-rats
#11
Binitha P Purushothaman, Ramadasan Kuttan
Pesticide carbofuran (Furadan) is known to be an inhibitor of nerve impulses by its inhibition of acetylcholinesterase. Oral administration of carbofuran also produced tissue damage as evidenced by the increased the activities of creatine kinase (CK), lactate dehydrogenase (LDH), and gamma glutamyl transferase (γ-GT) in tissues and serum. Against carbofuran, curcumin restored the activities of acetylcholinesterase, LDH, CK and γ- GT in animals. Curcumin also restored the reduced mitochondrial enzymes in rat liver caused by carbofuran...
2017: Journal of Environmental Pathology, Toxicology and Oncology
https://www.readbyqxmd.com/read/28604960/-analysis-of-uqcrb-gene-mutation-in-a-child-with-mitochondrial-complex-iii-deficiency
#12
Ting Zhang, Fang Hong, Guling Qian, Fan Tong, Xuelian Zhou, Xiaolei Huang, Rulai Yang, Xinwen Huang
OBJECTIVE: To delineate the clinical, biochemical and genetic mutational characteristics of a child with mitochondrial complex III deficiency. METHODS: Clinical information and results of auxiliary examination of the patient were analyzed. Next-generation sequencing of the mitochondrial genome and related nuclear genes was carried out. Suspected mutation was confirmed in both parents with Sanger sequencing. Heterozygous deletion was mapped with chromosomal microarray analysis and confirmed with real-time PCR...
June 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28602551/circulating-htra2-as-a-novel-biomarker-for-mitochondrial-induced-cardiomyocyte-apoptosis-and-ischemia-reperfusion-injury-in-st-segment-elevation-myocardial-infarction
#13
M Hortmann, S Robinson, M Mohr, D Haenel, M Mauler, D Stallmann, J Reinoehl, D Duerschmied, K Peter, C Bode, I Ahrens
BACKGROUND: Ischemia-reperfusion (I/R) injury in ST-segment elevation myocardial infarction (STEMI) significantly contributes to overall myocardial damage. As a consequence of I/R injury in the heart, the high-temperature requirement protein A2 (HtrA2) is released from the mitochondrial intermembrane space of cardiomyocytes to the cytoplasm, whereupon it induces apoptosis. METHODS: Serum was obtained from STEMI (n=37), non-ST-segment elevation myocardial infarction (NSTEMI) (n=20), stable coronary artery disease (CAD) (n=17) and patients with CAD excluded (n=9)...
September 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28582851/fenofibrate-protects-against-acute-myocardial-i-r-injury-in-rat-by-suppressing-mitochondrial-apoptosis-as-decreasing-cleaved-caspase-9-activation
#14
Qing Zhao, Zheng Cui, Yan Zheng, Qun Li, Changyuan Xu, Xueqi Sheng, Mei Tao, HuiXin Xu
BACKGROUND AND AIMS: Peroxisome proliferator-activated receptor-α (PPAR-α) activation has been reported to reduce myocardial ischemia-reperfusion (I/R) injury by inhibiting cell apoptosis. However, the antiapoptotic mechanism of PPAR-α is still unknown. Fenofibrate is a PPAR-α agonist In the present study, we investigate the effects and relevant mechanism of fenofibrate on experimental myocardial ischemia-reperfusion (I/R) injury in rats. METHODS: Adult male Wistar rats were pretreated with fenofibrate (80 mg/kg) daily for a period of 7 days...
July 4, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/28578259/the-protective-effects-of-compatibility-of-aconiti-lateralis-radix-praeparata-and-zingiberis-rhizoma-on-rats-with-heart-failure-by-enhancing-mitochondrial-biogenesis-via-sirt1-pgc-1%C3%AE-pathway
#15
Xiaohua Lu, Lu Zhang, Pengyan Li, Jiabo Wang, Ruisheng Li, Yinqiu Huang, Mingquan Wu, Houqin Zhou, Yang Li, Shizhang Wei, Kun Li, Haotian Li, Xuelin Zhou, Yanling Zhao, Xiaohe Xiao
Aconiti Lateralis Radix Praeparata ("Fuzi" in Chinese) in combination with Zingiberis Rhizoma ("Ganjiang" in Chinese) is commonly applied for the treatment of heart failure for thousands of years in China. However, its therapeutic mechanism is still poorly defined. This study aimed to investigate whether the compatibility of Fuzi and Ganjiang can protect rats with acute heart failure by enhancing mitochondrial biogenesis via Sirt1/PGC-1α signaling pathway. Hemodynamic parameters, including heart rate and left ventricular maximal rate of pressure rise and decline, were recorded in rats with acute heart failure induced by Propafenone hydrochloride...
August 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28559828/multisystem-disease-including-eosinophilia-and-progressive-hyper-creatine-kinase-emia-over-10-years-suggests-mitochondrial-disorder
#16
Josef Finsterer, Johannes Huber
BACKGROUND: Eosinophilia has not been reported as a manifestation of a mitochondrial disorder (MID). Here, we report a patient with clinical features suggesting a MID and permanent eosinophilia, multisystem disease, and progressive hyper-creatine-kinase (CK)-emia for at least 10 years. MATERIALS AND METHODS: Methods applied included a clinical exam, blood chemical investigations, electrophysiological investigations, imaging, and invasive cardiological investigations...
January 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/28542490/repeated-bouts-of-exhaustive-exercise-increase-circulating-cell-free-nuclear-and-mitochondrial-dna-without-development-of-tolerance-in-healthy-men
#17
Robert Stawski, Konrad Walczak, Piotr Kosielski, Pawel Meissner, Tomasz Budlewski, Gianluca Padula, Dariusz Nowak
OBJECTIVE: Acute single strenuous exercise increases circulating cell free DNA (cf DNA). We tested whether three repeated bouts of exhaustive exercise induced the cf DNA response without development of tolerance in healthy men. METHODS: Eleven average-trained men (age 34.0±5.2 years, body mass index 26.2±3.1 kg/m2, maximal oxygen consumption-VO2max 49.6±4.5 ml/kg*min) performed three treadmill exercise tests to exhaustion at speed corresponding to 70% VO2max separated by 72 hours of resting...
2017: PloS One
https://www.readbyqxmd.com/read/28529047/bezafibrate-prevents-mitochondrial-dysfunction-antioxidant-system-disturbance-glial-reactivity-and-neuronal-damage-induced-by-sulfite-administration-in-striatum-of-rats-implications-for-a-possible-therapeutic-strategy-for-sulfite-oxidase-deficiency
#18
Mateus Grings, Alana Pimentel Moura, Belisa Parmeggiani, Julia Tauana Pletsch, Gabriela Miranda Fernandez Cardoso, Pauline Maciel August, Cristiane Matté, Angela T S Wyse, Moacir Wajner, Guilhian Leipnitz
Sulfite accumulates in tissues of patients affected by sulfite oxidase (SO) deficiency, a neurometabolic disease characterized by seizures and progressive encephalopathy, often resulting in early death. We investigated the effects of sulfite on mitochondrial function, antioxidant system, glial reactivity and neuronal damage in rat striatum, as well as the potential protective effects of bezafibrate on sulfite-induced toxicity. Thirty-day-old rats were intrastriatally administered with sulfite (2μmol) or NaCl (2μmol; control) and euthanized 30min after injection for evaluation of biochemical parameters and western blotting, or 7days after injection for analysis of glial reactivity and neuronal damage...
May 18, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28521058/enhanced-expression-of-proteins-involved-in-energy-production-and-transfer-in-breast-muscle-of-pedigree-male-broilers-exhibiting-high-feed-efficiency
#19
W G Bottje, K Lassiter, S Dridi, N Hudson, B-W Kong
In cells with fluctuating energy demand (e.g., skeletal muscle), a transfer system of proteins across the inner and outer mitochondrial membranes links mitochondrial oxidative phosphorylation to cytosolic phosphorylated creatine (PCr) that serves as a phosphate reservoir for rapid repletion of cytosolic adenosine triphosphate (ATP). Crucial proteins of this energy transfer system include several creatine kinase (CK) isoforms found in the cytosol and mitochondria. In a recent proteomic study (Kong et al., 2016), several components of this system were up-regulated in high feed efficiency (FE) compared to low FE breast muscle; notably adenine nucleotide translocase (ANT), voltage dependent activated channel (VDAC), the brain isoform of creatine kinase (CK-B), and several proteins of the electron transport chain...
May 17, 2017: Poultry Science
https://www.readbyqxmd.com/read/28496033/joint-toxicity-of-fluoroquinolone-and-tetracycline-antibiotics-to-zebrafish-danio-rerio-based-on-biochemical-biomarkers-and-histopathological-observation
#20
Lihe Ding, Luxiu Zang, Yuna Zhang, Yuhuan Zhang, Xuedong Wang, Weiming Ai, Nani Ding, Huili Wang
Herein, we report on the joint toxicity of four fluoroquinolones and two tetracyclines (β-diketone antibiotics-DKAs) to zebrafish based on a series of toxicological endpoints and histopathological observations. A positive dose-dependence was observed in DKA-exposure groups with a 72-hpf EC50 of 130.3 mg/L for hatching rate, 120-hpf LC50 of 149.8 mg/L, and 120-hpf EC50 of 135.1 mg/L for malformation rate. When zebrafish at 60 dpf were exposed to a series of DKA concentrations (45, 60 and 90 mg/L) for 7, 14 and 21 days, creatine kinase and AChE activities were significantly induced, and intracellular malondialdehyde increased in all treatments except for the 45 mg/L treatment...
2017: Journal of Toxicological Sciences
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