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Mitochondrial creatine kinase

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https://www.readbyqxmd.com/read/29762537/the-cardioprotective-effect-of-metformin-in-doxorubicin-induced-cardiotoxicity-the-role-of-autophagy
#1
Rita Zilinyi, Attila Czompa, Andras Czegledi, Andrea Gajtko, Dora Pituk, Istvan Lekli, Arpad Tosaki
The molecular mechanisms underlying doxorubicin-induced cardiotoxicity are still being investigated, but are known to involve oxidative stress, mitochondrial dysfunction, and the dysregulation of autophagy. The objective of the current study was to examine the protective role of metformin and its effect on autophagy in doxorubicin-induced cardiotoxicity. Sprague⁻Dawley rats were divided into four groups at random. The doxorubicin-treated group received doxorubicin (3 mg/kg every second day) intraperitoneally...
May 15, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29747667/protective-effect-of-grape-seed-and-skin-extract-against-high-fat-diet-induced-dyshomeostasis-of-energetic-metabolism-in-rat-lung
#2
Mohamed El Ayed, Safwen Kadri, Maha Mabrouk, Ezzedine Aouani, Salem Elkahoui
BACKGROUND: Obesity is currently one of the major epidemics of this millennium and affects poeples throughout the world. It causes multiple systemic complications as it significantly interferes with respiratory function. OBJECTIVE: We aimed in the present work to study the effect of high fat diet (HFD) on lung oxidative stress and energy metabolism alterations, as well as the putative protection afforded by grape seed and skin extract (GSSE). METHODS: We started by characterizing the GSSE and its composition using gas chromatography coupled to mass spectrometry (GC-MS)...
May 10, 2018: Lipids in Health and Disease
https://www.readbyqxmd.com/read/29736620/cardioprotective-effect-of-zingerone-against-oxidative-stress-inflammation-and-apoptosis-induced-by-cisplatin-or-gamma-radiation-in-rats
#3
Ahmed F Soliman, Lobna M Anees, Doaa M Ibrahim
Despite their clinical benefits in cancer treatment, the deleterious effects on heart following chemo/radiotherapy are of increasing importance. Zingerone, a natural polyphenol, possesses multiple biological activities, such as antioxidant and anti-inflammatory. Thus, the current study was designed to assess the potential cardioprotective effects of zingerone against cisplatin or γ-radiation. Zingerone was given by intragastric intubation (25 mg/kg) daily for three successive weeks prior to the induction of cardiotoxicity using a single dose of cisplatin (20 mg/kg, i...
May 7, 2018: Naunyn-Schmiedeberg's Archives of Pharmacology
https://www.readbyqxmd.com/read/29731937/carnitine-palmitoyltransferase-ii-deficiency-cpt-ii-followed-by-rhabdomyolysis-and-acute-kidney-injury
#4
Nikola Gjorgjievski, Pavlina Dzekova-Vidimliski, Zvezdana Petronijevic, Gjulsen Selim, Petar Dejanov, Liljana Tozija, Aleksandar Sikole
BACKGROUND: Carnitine palmitoyltransferase II deficiency (CPT II) is an autosomal recessive disorder and the most common inherited disorder of mitochondrial long-chain fatty acid oxidation, characterised by attacks of myalgia and myoglobinuria. The most common "classic" myopathic form occurs in young adults and is characterised by recurrent episodes of rhabdomyolysis triggered by prolonged exercise, fasting or febrile illness. CASE PRESENTATION: We present a case of a 22-year-old Caucasian male admitted to our hospital with fever, dyspnea, fatigue, myalgia and dark urine (brown-coloured)...
April 15, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29721912/a-middle-eastern-founder-mutation-expands-the-genotypic-and-phenotypic-spectrum-of-mitochondrial-micu1-deficiency-a-report-of-13-patients
#5
Sara Musa, Wafaa Eyaid, Kimberli Kamer, Rehab Ali, Mariam Al-Mureikhi, Noora Shahbeck, Fatma Al Mesaifri, Nawal Makhseed, Zakkiriah Mohamed, Wafaa Ali AlShehhi, Vamsi K Mootha, Jane Juusola, Tawfeg Ben-Omran
MICU1 encodes a Ca2+ sensing, regulatory subunit of the mitochondrial uniporter, a selective calcium channel within the organelle's inner membrane. Ca2+ entry into mitochondria helps to buffer cytosolic Ca2+ transients and also activates ATP production within the organelle. Mutations in MICU1 have previously been reported in 17 children from nine families with muscle weakness, fatigue, normal lactate, and persistently elevated creatine kinase, as well as variable features that include progressive extrapyramidal signs, learning disabilities, nystagmus, and cataracts...
May 3, 2018: JIMD Reports
https://www.readbyqxmd.com/read/29718187/mutations-in-coa7-cause-spinocerebellar-ataxia-with-axonal-neuropathy
#6
Yujiro Higuchi, Ryuta Okunushi, Taichi Hara, Akihiro Hashiguchi, Junhui Yuan, Akiko Yoshimura, Kei Murayama, Akira Ohtake, Masahiro Ando, Yu Hiramatsu, Satoshi Ishihara, Hajime Tanabe, Yuji Okamoto, Eiji Matsuura, Takehiro Ueda, Tatsushi Toda, Sumimasa Yamashita, Kenichiro Yamada, Takashi Koide, Hiroaki Yaguchi, Jun Mitsui, Hiroyuki Ishiura, Jun Yoshimura, Koichiro Doi, Shinichi Morishita, Ken Sato, Masanori Nakagawa, Masamitsu Yamaguchi, Shoji Tsuji, Hiroshi Takashima
Several genes related to mitochondrial functions have been identified as causative genes of neuropathy or ataxia. Cytochrome c oxidase assembly factor 7 (COA7) may have a role in assembling mitochondrial respiratory chain complexes that function in oxidative phosphorylation. Here we identified four unrelated patients with recessive mutations in COA7 among a Japanese case series of 1396 patients with Charcot-Marie-Tooth disease (CMT) or other inherited peripheral neuropathies, including complex forms of CMT...
April 27, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29709859/gill-bioenergetics-dysfunction-and-oxidative-damage-induced-by-thiamethoxam-exposure-as-relevant-toxicological-mechanisms-in-freshwater-silver-catfish-rhamdia-quelen
#7
Matheus D Baldissera, Carine F Souza, Débora Seben, Letícia R Sippert, Joseania Salbego, Enio Marchesan, Renato Zanella, Bernardo Baldisserotto, Jaqueline I Golombieski
Thiamethoxam is a neonicotinoid pesticide utilized on a worldwide scale, it has been reported in freshwater ecosystems, and detected in fishery products. Nevertheless, there is a lack of information about thiamethoxam sublethal effects on the gills of freshwater fish, principally linked to energetic metabolism. In this context, creatine kinase (CK) is an enzyme of the phosphoryl transfer network that provides a temporal and spatial energy buffer to maintain cellular energy homeostasis in tissues with high energy requirements, such as gills...
April 27, 2018: Science of the Total Environment
https://www.readbyqxmd.com/read/29705043/using-1-h-2-o-mr-to-measure-and-map-sodium-pump-activity-in-vivo
#8
Charles S Springer
The cell plasma membrane Na+ ,K+ -ATPase [NKA] is one of biology's most [if not the most] significant enzymes. By actively transporting Na+ out [and K+ in], it maintains the vital trans-membrane ion concentration gradients and the membrane potential. The forward NKA reaction is shown in the Graphical Abstract [which is elaborated in the text]. Crucially, NKA does not operate in isolation. There are other transporters that conduct K+ back out of [II, Graphical Abstract] and Na+ back into [III, Graphical Abstract] the cell...
April 25, 2018: Journal of Magnetic Resonance
https://www.readbyqxmd.com/read/29691892/congenital-titinopathy-comprehensive-characterisation-and-pathogenic-insights
#9
Emily C Oates, Kristi J Jones, Sandra Donkervoort, Amanda Charlton, Susan Brammah, John E Smith, James S Ware, Kyle S Yau, Lindsay C Swanson, Nicola Whiffin, Anthony J Peduto, Adam Bournazos, Leigh B Waddell, Michelle A Farrar, Hugo A Sampaio, Hooi Ling Teoh, Phillipa J Lamont, David Mowat, Robin B Fitzsimons, Alastair J Corbett, Monique M Ryan, Gina L O'Grady, Sarah A Sandaradura, Roula Ghaoui, Himanshu B Joshi, Jamie L Marshall, Melinda A Nolan, Simranpreet Kaur, Jaya Punetha, Ana Töpf, Elizabeth Harris, Madhura Bakshi, Casie A Genetti, Minttu Marttila, Ulla Werlauff, Nathalie Streichenberger, Alan Pestronk, Ingrid Mazanti, Jason R Pinner, Carole Vuillerot, Carla Grosmann, Ana Camacho, Payam Mohassel, Meganne E Leach, A Reghan Foley, Diana Bharucha-Goebel, James Collins, Anne M Connolly, Heather R Gilbreath, Susan T Iannaccone, Diana Castro, Beryl B Cummings, Richard I Webster, Leïla Lazaro, John Vissing, Sandra Coppens, Nicolas Deconinck, Ho-Ming Luk, Neil H Thomas, Nicola C Foulds, Marjorie A Illingworth, Sian Ellard, Catriona A McLean, Rahul Phadke, Gianina Ravenscroft, Nanna Witting, Peter Hackman, Isabelle Richard, Sandra T Cooper, Erik-Jan Kamsteeg, Eric P Hoffman, Kate Bushby, Volker Straub, Bjarne Udd, Ana Ferreiro, Kathryn N North, Nigel F Clarke, Monkol Lek, Alan H Beggs, Carsten G Bönnemann, Daniel G MacArthur, Henk Granzier, Mark R Davis, Nigel G Laing
OBJECTIVE: Comprehensive clinical characterisation of congenital titinopathy to facilitate diagnosis and management of this important emerging disorder. METHODS: Using massively parallel sequencing we identified 30 patients from 27 families with two pathogenic nonsense, frameshift and/or splice site TTN mutations in trans. We then undertook a detailed analysis of the clinical, histopathology and imaging features of these patients. RESULTS: All patients had prenatal- or early-onset hypotonia and/or congenital contractures...
April 25, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29679541/treatment-with-d-%C3%AE-hydroxybutyrate-protects-heart-from-ischemia-reperfusion-injury-in-mice
#10
Yongsheng Yu, Yunhua Yu, Yuefan Zhang, Zhigang Zhang, Weishuai An, Xianxian Zhao
The present study was designed to examine the protection of D-β-hydroxybutyrate (BHB) against ischemia/reperfusion (I/R) injury in heart and investigate its underlying mechanism. Male adult mice were exposed to 30min of ischemia and 24h of reperfusion. Osmotic pumps were implanted subcutaneously 5min before reperfusion for continuous delivery of the exogenous BHB (1.6mmol/kg/24h). Treatment with BHB reduced infarct size and levels of cardiac troponin I, creatine kinase and lactate dehydrogenase in serum, attenuated apoptosis in myocardium, and preserved cardiac function of I/R mice...
April 18, 2018: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29630131/butorphanol-attenuates-myocardial-ischemia-reperfusion-injury-through-inhibiting-mitochondria-mediated-apoptosis-in-mice
#11
L-H Huang, J Li, J-P Gu, M-X Qu, J Yu, Z-Y Wang
OBJECTIVE: To investigate the role of the opioid receptors agonist butorphanol on mice myocardial ischemia reperfusion (I/R) injury. MATERIALS AND METHODS: The left anterior descending of coronary artery was ligatured for 30 min and then reperfusion for 6 h was performed to mimic the mouse myocardial I/R injury. All mice were randomly divided into three groups: sham group, I/R group and I/R + butorphanol group. Blood samples were collected for the measurement of cardiac troponin I (CTnI) and creatine kinase MB (CK-MB) levels...
March 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29588219/exercise-induced-reductions-in-mitochondrial-adp-sensitivity-contribute-to-the-induction-of-gene-expression-and-mitochondrial-biogenesis-through-enhanced-mitochondrial-h-2-o-2-emission
#12
Paula M Miotto, Graham P Holloway
Acute exercise rapidly induces mitochondrial gene expression, however, the intracellular events regulating this process remain incompletely understood. The purpose of this study was to determine whether reductions in mitochondrial ADP sensitivity during exercise have a biological role in regulating mitochondrial-derived reactive oxygen species (ROS) production and the induction of mitochondrial biogenesis. Mitochondrial creatine kinase wildtype (WT) and knockout (KO) mice have divergent responses in ADP sensitivity during exercise, and we therefore used these mice to determine the relationship between mitochondrial ADP sensitivity, ROS production, and mitochondrial adaptations to exercise...
March 24, 2018: Mitochondrion
https://www.readbyqxmd.com/read/29584613/effect-of-l-carnitine-on-the-skeletal-muscle-contractility-in-simvastatin-induced-myopathy-in-rats
#13
Mohammad Ghalwash, Ahlam Elmasry, Nabil El-Adeeb
BACKGROUND: Statins therapy is effective in the prevention of cardiovascular events. However, its use is associated with skeletal muscle myopathy, which may be severe enough to discontinue statin therapy, thus exposing patients to more morbidity and mortality. This study was conducted to assess the effect of L-carnitine on the skeletal muscle contractility in a rat model of statin-induced myopathy and to clarify its possible mechanisms. METHODS: Twenty-one female Sprague Dawley rats were used throughout this study...
March 27, 2018: Journal of Basic and Clinical Physiology and Pharmacology
https://www.readbyqxmd.com/read/29552494/open-label-clinical-trial-of-bezafibrate-treatment-in-patients-with-fatty-acid-oxidation-disorders-in-japan
#14
Kenji Yamada, Hideaki Shiraishi, Eishin Oki, Mika Ishige, Toshiyuki Fukao, Yusuke Hamada, Norio Sakai, Fumihiro Ochi, Asami Watanabe, Sanae Kawakami, Kazuyo Kuzume, Kenji Watanabe, Koji Sameshima, Kiyotaka Nakamagoe, Akira Tamaoka, Naoko Asahina, Saki Yokoshiki, Takashi Miyakoshi, Kota Ono, Koji Oba, Toshiyuki Isoe, Hiroshi Hayashi, Seiji Yamaguchi, Norihiro Sato
Introduction: Fatty acid oxidation disorders (FAODs) are rare diseases caused by defects in mitochondrial fatty acid oxidation (FAO) enzymes. While the efficacy of bezafibrate, a peroxisome proliferator-activated receptor agonist, on the in vitro FAO capacity has been reported, the in vivo efficacy remains controversial. Therefore, we conducted a clinical trial of bezafibrate in Japanese patients with FAODs. Materials and methods: This trial was an open-label, non-randomized, and multicenter study of bezafibrate treatment in 6 patients with very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency and 2 patients with carnitine palmitoyltransferase-II (CPT-2) deficiency (median age, 8...
June 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29552206/protective-effect-of-berberine-on-acute-cardiomyopathy-associated-with-doxorubicin-treatment
#15
Chen Xiong, Yan-Zhao Wu, Yu Zhang, Zi-Xiao Wu, Xue-Yan Chen, Ping Jiang, Hui-Cai Guo, Ke-Rang Xie, Ke-Xin Wang, Su-Wen Su
Doxorubicin (DOX) is a potent and broad-spectrum anthracycline chemotherapeutic agent, but dose-dependent cardiotoxic side effects limit its clinical application. This toxicity is closely associated with the generation of reactive oxygen species (ROS) radical during DOX metabolism. The present study investigated the effects of Berberine (Ber) on DOX-induced acute cardiac injury in a rat model and analysed its mechanism in cardiomyocytes in vitro . Serum creatine kinase (CK), creatine kinase isoenzyme (CK-MB) and malondialdehyde (MDA) levels were significantly increased in the DOX group compared with the control group...
April 2018: Oncology Letters
https://www.readbyqxmd.com/read/29546539/changes-in-the-cerebral-phosphotransfer-network-impair-energetic-homeostasis-in-an-aflatoxin-b-1-contaminated-diet
#16
Matheus D Baldissera, Carine F Souza, Carla Cristina Zeppenfeld, Sharine Descovi, Aleksandro S da Silva, Bernardo Baldisserotto
The phosphotransfer network system, through the enzymes creatine kinase (CK), adenylate kinase (AK), and pyruvate kinase (PK), contributes to efficient intracellular energetic communication between cellular adenosine triphosphate (ATP) consumption and production in tissues with high energetic demand, such as cerebral tissue. Thus, the aim of this study was to evaluate whether aflatoxin B1 (AFB1 ) intoxication in diet negatively affects the cerebral phosphotransfer network related to impairment of cerebral ATP levels in silver catfish (Rhamdia quelen)...
March 15, 2018: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/29532476/effect-of-resistance-training-and-protein-intake-pattern-on-myofibrillar-protein-synthesis-and-proteome-kinetics-in-older-men-in-energy-restriction
#17
Caoileann H Murphy, Mahalakshmi Shankaran, Tyler A Churchward-Venne, Cameron J Mitchell, Nathan M Kolar, Louise M Burke, John A Hawley, Amira Kassis, Leonidas G Karagounis, Kelvin Li, Chelsea King, Marc Hellerstein, Stuart M Phillips
KEY POINTS: Strategies to enhance the loss of fat while preserving muscle mass during energy restriction are of great importance to prevent sarcopenia in overweight older adults. We show for the first time that the integrated rate of synthesis of numerous individual contractile, cytosolic and mitochondrial skeletal muscle proteins was increased by resistance training (RT) and unaffected by dietary protein intake pattern during energy restriction in free-living, obese older men. We observed a correlation between the synthetic rates of skeletal muscle-derived proteins obtained in serum (creatine kinase M-type, carbonic anhydrase 3) and the synthetic rates of proteins obtained via muscle sampling; and that the synthesis rates of these proteins in serum revealed the stimulatory effects of RT...
March 12, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/29509881/over-expression-of-mitochondrial-creatine-kinase-in-the-murine-heart-improves-functional-recovery-and-protects-against-injury-following-ischaemia-reperfusion
#18
Hannah J Whittington, Philip J Ostrowski, Debra J McAndrew, Fang Cao, Andrew Shaw, Thomas R Eykyn, Hannah Lake, Jack Tyler, Jurgen E Schneider, Stefan Neubauer, Sevasti Zervou, Craig A Lygate
Aims: Mitochondrial creatine kinase (MtCK) couples ATP production via oxidative phosphorylation to phosphocreatine in the cytosol, which acts as a mobile energy store available for regeneration of ATP at times of high demand. We hypothesised that elevating MtCK would be beneficial in ischaemia-reperfusion (I/R) injury. Methods and Results: Mice were created overexpressing the sarcomeric MtCK gene with αMHC promoter at the Rosa26 locus (MtCK-OE) and compared with wild-type (WT) littermates...
March 2, 2018: Cardiovascular Research
https://www.readbyqxmd.com/read/29471134/citrobacter-freundii-impairs-the-phosphoryl-transfer-network-in-the-gills-of-rhamdia-quelen-impairment-of-bioenergetics-homeostasis
#19
Matheus D Baldissera, Carine F Souza, Guerino B Junior, Karen Luise S Moreira, Marcelo L da Veiga, Maria Izabel U M da Rocha, Bernardo Baldisserotto
The precise coupling of spatially separated intracellular adenosine triphosphate (ATP)-producing and ATP-consuming, catalyzed by creatine kinase (CK), adenylate kinase (AK), and pyruvate kinase (PK), is a critical process in the bioenergetics of tissues with high energy demand, such as the branchial tissue. The effects of Citrobacter freundii infection on gills remain poorly understood, limited only to histopathological studies. Thus, the aim of this study was to evaluate whether experimental infection by C...
April 2018: Microbial Pathogenesis
https://www.readbyqxmd.com/read/29465611/mitochondrial-dna-deletions-with-low-level-heteroplasmy-in-adult-onset-myopathy
#20
Doris G Leung, Julie S Cohen, Elizabeth Harlan Michelle, Renkui Bai, Andrew L Mammen, Lisa Christopher-Stine
We report the cases of 2 patients who presented to our Myositis Center with myalgias and elevated creatine kinase levels. Muscle biopsy showed pathological features consistent with mitochondrial myopathy. In both cases, a single large deletion in mitochondrial DNA at low-level heteroplasmy was identified by next-generation sequencing in muscle tissue. In 1 case, the deletion was identified in muscle tissue but not blood. In both cases, the deletion was only identified on next-generation sequencing of muscle mitochondrial DNA and missed on array comparative genome hybridization testing...
March 2018: Journal of Clinical Neuromuscular Disease
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