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Mitochondrial creatine kinase

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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
#1
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 the brains 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 14, 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
#2
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
#3
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
#4
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
#5
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)...
May 24, 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
#6
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...
May 12, 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
#7
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...
June 1, 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
#8
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
#9
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
#10
Mateus Grings, Alana Pimentel Moura, Belisa Parmeggiani, Julia Tauna 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
#11
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
#12
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
https://www.readbyqxmd.com/read/28489755/kennedy-disease-with-difficulty-in-differential-diagnosis-a-case-report
#13
Yating Chen, Peng Luo, Zhongli Li, Hengping Hu, Duobin Wu, Tingting Xu, Xingzuo Wang, Haiting Xie
RATIONALE: Kennedy disease (KD) is also known as spinal bulbar muscular dystrophy. As KD has similar symptoms with most neuromuscular diseases, so it is difficult to make a rapid diagnosis clinically. PATIENT CONCERNS: We report a case of a 43-year-old male with progressive limb proximal weakness without family history. Physical examination showed gynecomastia, erectile dysfunction, bilateral tendon reflex and quadriceps weakness, and tongue muscle atrophy. DIAGNOSES: Laboratory examination found increased creatine kinase, impaired glucose tolerance, and abnormal lactic acid values...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28479248/effects-of-non-invasive-remote-ischemic-conditioning-on-rehabilitation-after-myocardial-infarction
#14
Meng-Yao An, Yan Li, Wen-Hua Chen, Ying Zhang, Yan-Na Wu, Kai Sun, Ying-Ying Pan, Yong-Qiang Yin, Jian-Shi Lou
Recent studies have demonstrated that remote ischemic conditioning (RIC) creates cardioprotection against ischemia/reperfusion injury and myocardial infarction (MI); however, the effects of non-invasive remote ischemic conditioning (nRIC) on prognosis and rehabilitation after MI (post-MI) remain unknown. We successfully established MI models involving healthy adult male Sprague-Dawley rats. The nRIC group repeatedly underwent 5 min of ischemia and 5 min of reperfusion in the left hind limb for three cycles every other day until weeks 4, 6, and 8 after MI...
May 4, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28417315/disruption-of-energy-transfer-and-redox-status-by-sulfite-in-hippocampus-striatum-and-cerebellum-of-developing-rats
#15
Leonardo de Moura Alvorcem, Mateus Struecker da Rosa, Nícolas Manzke Glänzel, Belisa Parmeggiani, Mateus Grings, Felipe Schmitz, Angela T S Wyse, Moacir Wajner, Guilhian Leipnitz
Patients with sulfite oxidase (SO) deficiency present severe brain abnormalities, whose pathophysiology is not yet elucidated. We evaluated the effects of sulfite and thiosulfate, metabolites accumulated in SO deficiency, on creatine kinase (CK) activity, mitochondrial respiration and redox status in hippocampus, striatum and cerebellum of developing rats. Our in vitro results showed that sulfite and thiosulfate decreased CK activity, whereas sulfite also increased malondialdehyde (MDA) levels in all brain structures evaluated...
April 17, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28408707/admixture-mapping-of-subclinical-atherosclerosis-and-subsequent-clinical-events-among-african-americans-in-2-large-cohort-studies
#16
Aditi Shendre, Howard Wiener, Marguerite R Irvin, Degui Zhi, Nita A Limdi, Edgar T Overton, Christina L Wassel, Jasmin Divers, Jerome I Rotter, Wendy S Post, Sadeep Shrestha
BACKGROUND: Local ancestry may contribute to the disproportionate burden of subclinical and clinical cardiovascular disease among admixed African Americans compared with other populations, suggesting a rationale for admixture mapping. METHODS AND RESULTS: We estimated local European ancestry (LEA) using Local Ancestry inference in adMixed Populations using Linkage Disequilibrium method (LAMP-LD) and evaluated the association with common carotid artery intima-media thickness (cCIMT) using multivariable linear regression analysis among 1554 African Americans from MESA (Multi-Ethnic Study of Atherosclerosis)...
April 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28293876/changes-in-the-mitochondrial-function-and-in-the-efficiency-of-energy-transfer-pathways-during-cardiomyocyte-aging
#17
Kersti Tepp, Marju Puurand, Natalja Timohhina, Jasper Adamson, Aleksandr Klepinin, Laura Truu, Igor Shevchuk, Vladimir Chekulayev, Tuuli Kaambre
The role of mitochondria in alterations that take place in the muscle cell during healthy aging is a matter of debate during recent years. Most of the studies in bioenergetics have a focus on the model of isolated mitochondria, while changes in the crosstalk between working myofibrils and mitochondria in senescent cardiomyocytes have been less studied. The aim of our research was to investigate the modifications in the highly regulated ATP production and energy transfer systems in heart cells in old rat cardiomyocytes...
March 14, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28293875/angiotensin-1-7-facilitates-cardioprotection-of-ischemic-preconditioning-on-ischemia-reperfusion-challenged-rat-heart
#18
Pradeepkant Pachauri, Debapriya Garabadu, Ahsas Goyal, Prabhat Kumar Upadhyay
Ischemic preconditioning (IPC) is one of the most promising strategies used to protect the myocardium from ischemia-reperfusion injury. Ang (1-7) exhibits cardioprotective activity; however, its therapeutic potential on IPC-induced cardioprotection has not been reported in ischemia-reperfusion injury till date. Therefore, the first set of experiment was designed to evaluate the direct effect of Ang (1-7), in perfusion medium, on cardioprotective activity of IPC in rat heart challenged to ischemia-reperfusion injury...
March 14, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28290047/adaptation-to-chronic-continuous-hypoxia-potentiates-akt-hk2-anti-apoptotic-pathway-during-brief-myocardial-ischemia-reperfusion-insult
#19
David Kolar, Milada Gresikova, Petra Waskova-Arnostova, Barbara Elsnicova, Jana Kohutova, Daniela Hornikova, Pavel Vebr, Jan Neckar, Tereza Blahova, Dita Kasparova, Jiri Novotny, Frantisek Kolar, Olga Novakova, Jitka M Zurmanova
Adaptation to chronic hypoxia represents a potential cardioprotective intervention reducing the extent of acute ischemia/reperfusion (I/R) injury, which is a major cause of death worldwide. The main objective of this study was to investigate the anti-apoptotic Akt/hexokinase 2 (HK2) pathway in hypoxic hearts subjected to I/R insult. Hearts isolated from male Wistar rats exposed either to continuous normobaric hypoxia (CNH; 10% O2) or to room air for 3 weeks were perfused according to Langendorff and subjected to 10 min of no-flow ischemia and 10 min of reperfusion...
March 13, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28284597/pseudomonas-aeruginosa-strain-pa01-impairs-enzymes-of-the-phosphotransfer-network-in-the-gills-of-rhamdia-quelen
#20
Matheus D Baldissera, Carine F Souza, Roberto C V Santos, Lenita M Stefani, Karen Luise S Moreira, Marcelo L da Veiga, Maria Izabel U M da Rocha, Bernardo Baldisserotto
Integration of mitochondria with cytosolic ATP-consuming/ATP-sensing and substrate supply processes is critical for gills bioenergetics, since this tissue plays an important role in the respiratory energy metabolism. The effects of bacterial infection on gills remain poorly understood, limited only to histopathological analyses. Thus, the aim of this study was to investigate whether experimental infection by Pseudomonas aeruginosa strain PA01 alters the enzymes of the phosphoryltransfer network (adenylate kinase (AK), pyruvate kinase (PK) and cytosolic and mitochondrial creatine kinase (CK)) in gills of silver catfish (Rhamdia quelen)...
March 2017: Veterinary Microbiology
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