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https://www.readbyqxmd.com/read/27907123/mice-hemizygous-for-a-pathogenic-mitofusin-2-allele-exhibit-hind-limb-foot-gait-deficits-and-phenotypic-perturbations-in-nerve-and-muscle
#1
Peter Bannerman, Travis Burns, Jie Xu, Laird Miers, David Pleasure
Charcot-Marie-Tooth disease type 2A (CMT2A), the most common axonal form of hereditary sensory motor neuropathy, is caused by mutations of mitofusin-2 (MFN2). Mitofusin-2 is a GTPase required for fusion of mitochondrial outer membranes, repair of damaged mitochondria, efficient mitochondrial energetics, regulation of mitochondrial-endoplasmic reticulum calcium coupling and axonal transport of mitochondria. We knocked T105M MFN2 preceded by a loxP-flanked STOP sequence into the mouse Rosa26 locus to permit cell type-specific expression of this pathogenic allele...
2016: PloS One
https://www.readbyqxmd.com/read/27895764/mitofusin-2-prevents-skeletal-muscle-wasting-in-cancer-cachexia
#2
Qiu-Lei Xi, Bo Zhang, Yi Jiang, Hai-Sheng Zhang, Qing-Yang Meng, Ying Chen, Yu-Song Han, Qiu-Lin Zhuang, Jun Han, Hai-Yu Wang, Jing Fang, Guo-Hao Wu
Cancer cachexia remains a leading cause of morbidity and mortality worldwide, despite extensive research and clinical trials. The prominent clinical feature of cancer cachexia is the continuous loss of skeletal muscle that cannot be fully reversed by conventional nutritional support, and that leads to progressive functional impairment. The mechanism underlying muscle loss in patients with cachexia is poorly understood. The present study analyzed 21 cancer patients with or without cachexia, and demonstrated that mitofusin-2 (Mfn2) was downregulated in the rectus abdominis of patients with cachexia, which was associated with body weight loss...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27862672/clinical-and-genetic-spectra-of-charcot-marie-tooth-disease-in-chinese-han-patients
#3
Bo Sun, Zhaohui Chen, Li Ling, Fei Yang, Xusheng Huang
Charcot-Marie-Tooth disease (CMT) is a common hereditary motor and sensory neuropathy. Epidemiological data for Chinese CMT patients are few. This study aimed to analyze the electrophysiological and genetic characteristics of Chinese Han patients. 106 unrelated patients with the clinical diagnosis of CMT were included. Clinical examination, nerve conduction studies (NCS), next-generation sequencing (NGS), and bioinformatic analyses were performed. Genetic testing was performed for 82 patients; 27 (33%) patients carried known CMT-associated gene mutations...
November 8, 2016: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27847330/altered-mitochondrial-quality-control-signaling-in-muscle-of-old-gastric-cancer-patients-with-cachexia
#4
Emanuele Marzetti, Maria Lorenzi, Francesco Landi, Anna Picca, Fausto Rosa, Fabiana Tanganelli, Marco Galli, Giovanni Battista Doglietto, Fabio Pacelli, Matteo Cesari, Roberto Bernabei, Riccardo Calvani, Maurizio Bossola
Mitochondrial dysfunction is involved in the loss of muscle featuring both aging and cancer cachexia (CC). Whether mitochondrial quality control (MQC) is altered in skeletal myocytes of old patients with CC is unclear. The present investigation therefore sought to preliminarily characterize MQC pathways in muscle of old gastric cancer patients with cachexia. The study followed a case-control cross-sectional design. Intraoperative biopsies of the rectus abdominis muscle were obtained from 18 patients with gastric adenocarcinoma (nine with CC and nine non-cachectic) and nine controls, and assayed for the expression of a set of MQC mediators...
November 12, 2016: Experimental Gerontology
https://www.readbyqxmd.com/read/27847271/oleuropein-improves-mitochondrial-function-to-attenuate-oxidative-stress-by-activating-the-nrf2-pathway-in-the-hypothalamic-paraventricular-nucleus-of-spontaneously-hypertensive-rats
#5
Wenyan Sun, Xin Wang, Chen Hou, Liang Yang, Hongbao Li, Jing Guo, Chanjuan Huo, Molin Wang, Yuwang Miao, Jiankang Liu, Yuming Kang
Hypertension is associated with increased reactive oxygen species (ROS) production in the paraventricular nucleus (PVN) of the hypothalamus. Oleuropein (OL) has a variety of biochemical roles, including antihypertensive and antioxidative functions. However, there have been few reports on the effects of OL on oxidative stress in the PVN on hypertension. In spontaneously hypertensive rats (SHR), eight-week administration of 60 mg/kg/day of OL significantly reduced blood pressure, pro-inflammatory cytokines and the expression of components of the renin-angiotensin system (RAS) compared with SHR rats treated with saline...
November 12, 2016: Neuropharmacology
https://www.readbyqxmd.com/read/27837193/intact-initiation-of-autophagy-and-mitochondrial-fission-by-acute-exercise-in-skeletal-muscle-of-patientswith-type-2-diabetes
#6
Rikke Kruse, Andreas J T Pedersen, Jonas M Kristensen, Stine J Petersson, Jørgen F P Wojtaszewski, Kurt Højlund
AIMS: Type 2 diabetes (T2D) is characterized by insulin resistance, mitochondrial dysregulation, and, in some studies, exercise resistance in skeletal muscle. Regulation of autophagy and mitochondrial dynamics during exercise and recovery is important for skeletal muscle homeostasis, and these responses may be altered in T2D. MATERIALS AND METHODS: We examined the effect of acute exercise on markers of autophagy and mitochondrial fusion and fission in skeletal muscle biopsies from patients with T2D (n=13) and weight-matched controls (n=14) before, immediately after and 3h after an acute bout of exercise...
November 11, 2016: Clinical Science (1979-)
https://www.readbyqxmd.com/read/27833818/sevoflurane-postconditioning-attenuates-cardiomyocyte-hypoxia-reoxygenation-injury-via-restoring-mitochondrial-morphology
#7
Jin Yu, Jianjiang Wu, Peng Xie, Yiliyaer Maimaitili, Jiang Wang, Zhengyuan Xia, Feng Gao, Xing Zhang, Hong Zheng
BACKGROUND: Anesthetic postconditioning is a cellular protective approach whereby exposure to a volatile anesthetic renders a tissue more resistant to subsequent ischemic/reperfusion event. Sevoflurane postconditioning (SPostC) has been shown to exert cardioprotection against ischemia/reperfusion injury, but the underlying mechanism is unclear. We hypothesized that SPostC protects cardiomyocytes against hypoxia/reoxygenation (H/R) injury by maintaining/restoring mitochondrial morphological integrity, a critical determinant of cell fate...
2016: PeerJ
https://www.readbyqxmd.com/read/27830717/effect-of-heme-oxygenase-1-on-mitofusin-1-protein-in-lps-induced-ali-ards-in-rats
#8
Jianbo Yu, Ying Wang, Zhen Li, Shuan Dong, Dan Wang, Lirong Gong, Jia Shi, Yuan Zhang, Daquan Liu, Rui Mu
Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is a common and important oxidative stress in the lung. Mitochondrial fusion responds to the normal morphology and function of cells and is finely regulated by mitochondrial fusion proteins, such as mitofusin-1 protein (Mfn1), mitofusin-2 protein (Mfn2) and optical atrophy 1 (OPA1). Additionally, Mfn1 has been identified as the most important protein in mitochondrial fusion. Heme oxygenase-1 (HO-1) is a stress-inducible protein that plays a critical role in protecting against oxidative stress...
November 10, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27822413/mir-106b-mediated-mfn2-suppression-is-critical-for-pkm2-induced-mitochondrial-fusion
#9
Haili Wu, Zhuoyu Li, Yingying Wang, Peng Yang, Zongrui Li, Hanqing Li, Changxin Wu
Cancer cells preferentially metabolize glucose through aerobic glycolysis. This phenomenon, known as the Warburg effect, is a characteristic of glucose metabolism in cancer cells. PKM2 is reported to imply an important role in glycolysis. However, whether and how PKM2 can cause mitochondrial dysfunction, then subsequently forcing cancer cells using glycolysis instead of oxidation phosphorylation is poorly understood. Here we reported that overexpression of PKM2 disrupted mitochondrial dynamics by enhancing fusion...
2016: American Journal of Cancer Research
https://www.readbyqxmd.com/read/27706887/increased-mitochondrial-fusion-in-a-autosomal-recessive-cmt2a-family-with-mitochondrial-gtpase-mitofusin-2-mutations
#10
Philippe Codron, Arnaud Chevrollier, Mariame Selma Kane, Andoni Echaniz-Laguna, Philippe Latour, Pascal Reynier, Dominique Bonneau, Christophe Verny, Vincent Procaccio, Guy Lenaers, Julien Cassereau
Charcot-Marie-Tooth type 2A disease (CMT2A) is an inherited peripheral neuropathy mainly caused by mutations in the MFN2 gene coding for the mitochondrial fusion protein Mitofusin 2. Although the disease is mainly inherited in a dominant fashion, few cases of early-onset autosomal recessive CMT2A (AR-CMT2A) have been reported in recent years. In this study, we characterized the structure of mitochondrial network in cultured primary fibroblasts obtained from AR-CMT2A family members. The patient-derived cells showed an increase of the mitochondrial fusion with large connected networks and an increase of the mitochondrial volume...
October 5, 2016: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27693395/mir-195-dependent-roles-of-mitofusin2-in-the-mitochondrial-dysfunction-of-hippocampal-neurons-in-samp8-mice
#11
Rui Zhang, Huimin Zhou, Lei Jiang, Yueran Mao, Ximing Cui, Bing Xie, Dongsheng Cui, Hui Wang, Qingfu Zhang, Shunjiang Xu
Abnormal gene expression, including mRNAs, and microRNAs (miRNA), have been identified in the development of Alzheimer's disease (AD). Although mitofusin2 (mfn2) has been found to be down-regulated in the neurons from hippocampus and cortex in AD patients, little is known about its roles and the regulatory mechanisms in the pathogenesis of AD. This study was performed to investigate the roles of mfn2 protein and its upstream regulatory mechanism in the progression of AD using a senescence accelerated mouse prone-8 (SAMP8) model...
September 29, 2016: Brain Research
https://www.readbyqxmd.com/read/27684054/high-glucose-concentration-abrogates-sevoflurane-postconditioning-cardioprotection-by-advancing-mitochondrial-fission-but-dynamin-related-protein-1-inhibitor-restores-these-effects
#12
Jin Yu, Yiliyaer Maimaitili, Peng Xie, Jianjiang Wu, Jiang Wang, Yining Yang, Haiping Ma, Hong Zheng
AIM: Hyperglycaemia-induced cell injury is a primary cause of cardiovascular complications in diabetic patients. In vivo studies demonstrated that sevoflurane postconditioning (SpostC) was cardioprotective against ischaemia/reperfusion injury, which was blocked by hyperglycaemia. This study investigated whether high glucose concentration abrogated SpostC cardioprotection in vitro by advancing mitochondrial fission and whether mitochondrial division inhibitor-1 (Mdivi-1) restored SpostC cardioprotection in cultured primary neonatal rat cardiomyocytes (NCMs)...
September 29, 2016: Acta Physiologica
https://www.readbyqxmd.com/read/27677309/protective-effects-of-reduced-dynamin-related-protein-1-against-amyloid-beta-induced-mitochondrial-dysfunction-and-synaptic-damage-in-alzheimer-s-disease
#13
Maria Manczak, Ramesh Kandimalla, David Fry, Hiromi Sesaki, P Hemachandra Reddy
The purpose of our study was to understand the protective effects of reduced expression of dynamin-related protein (Drp1) against amyloid beta (Aβ) induced mitochondrial and synaptic toxicities in Alzheimer's disease (AD) progression and pathogenesis. Our recent molecular and biochemical studies revealed that impaired mitochondrial dynamics - increased mitochondrial fragmentation and decreased fusion - in neurons from autopsy brains of AD patients and from transgenic AD mice and neurons expressing Aβ, suggesting that Aβ causes mitochondrial fragmentation in AD...
September 27, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27647893/critical-reappraisal-confirms-that-mitofusin-2-is-an-endoplasmic-reticulum-mitochondria-tether
#14
Deborah Naon, Marta Zaninello, Marta Giacomello, Tatiana Varanita, Francesca Grespi, Sowmya Lakshminaranayan, Annalisa Serafini, Martina Semenzato, Stephanie Herkenne, Maria Isabel Hernández-Alvarez, Antonio Zorzano, Diego De Stefani, Gerald W Dorn, Luca Scorrano
The discovery of the multiple roles of mitochondria-endoplasmic reticulum (ER) juxtaposition in cell biology often relied upon the exploitation of Mitofusin (Mfn) 2 as an ER-mitochondria tether. However, this established Mfn2 function was recently questioned, calling for a critical re-evaluation of Mfn2's role in ER-mitochondria cross-talk. Electron microscopy and fluorescence-based probes of organelle proximity confirmed that ER-mitochondria juxtaposition was reduced by constitutive or acute Mfn2 deletion...
October 4, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27640307/recurrent-de-novo-and-biallelic-variation-of-atad3a-encoding-a-mitochondrial-membrane-protein-results-in-distinct-neurological-syndromes
#15
Tamar Harel, Wan Hee Yoon, Caterina Garone, Shen Gu, Zeynep Coban-Akdemir, Mohammad K Eldomery, Jennifer E Posey, Shalini N Jhangiani, Jill A Rosenfeld, Megan T Cho, Stephanie Fox, Marjorie Withers, Stephanie M Brooks, Theodore Chiang, Lita Duraine, Serkan Erdin, Bo Yuan, Yunru Shao, Elie Moussallem, Costanza Lamperti, Maria A Donati, Joshua D Smith, Heather M McLaughlin, Christine M Eng, Magdalena Walkiewicz, Fan Xia, Tommaso Pippucci, Pamela Magini, Marco Seri, Massimo Zeviani, Michio Hirano, Jill V Hunter, Myriam Srour, Stefano Zanigni, Richard Alan Lewis, Donna M Muzny, Timothy E Lotze, Eric Boerwinkle, Richard A Gibbs, Scott E Hickey, Brett H Graham, Yaping Yang, Daniela Buhas, Donna M Martin, Lorraine Potocki, Claudio Graziano, Hugo J Bellen, James R Lupski
ATPase family AAA-domain containing protein 3A (ATAD3A) is a nuclear-encoded mitochondrial membrane protein implicated in mitochondrial dynamics, nucleoid organization, protein translation, cell growth, and cholesterol metabolism. We identified a recurrent de novo ATAD3A c.1582C>T (p.Arg528Trp) variant by whole-exome sequencing (WES) in five unrelated individuals with a core phenotype of global developmental delay, hypotonia, optic atrophy, axonal neuropathy, and hypertrophic cardiomyopathy. We also describe two families with biallelic variants in ATAD3A, including a homozygous variant in two siblings, and biallelic ATAD3A deletions mediated by nonallelic homologous recombination (NAHR) between ATAD3A and gene family members ATAD3B and ATAD3C...
October 6, 2016: American Journal of Human Genetics
https://www.readbyqxmd.com/read/27640178/upregulated-mir-17-regulates-hypoxia-mediated-human-pulmonary-artery-smooth-muscle-cell-proliferation-and-apoptosis-by-targeting-mitofusin-2
#16
Zheng Lu, Sujun Li, Shunxin Zhao, Xianen Fa
BACKGROUND Pulmonary arterial hypertension (PAH) is a fatal disease characterized by impaired regulation of pulmonary artery vascular growth and remodeling. Aberrant expression of miR-17 has been shown to be involved in the pathogenesis of PAH, but its underlying molecular mechanism has not been elucidated. MATERIAL AND METHODS Mitofusin 2 (MFN2) expression was determined by qRT-PCR. The protein expression levels of MFN2, proliferating cell nuclear antigen (PCNA), and pro-apoptotic protein cleaved Caspase-3 were measured using Western blot analysis...
2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/27634647/reduced-dynamin-related-protein-1-protects-against-phosphorylated-tau-induced-mitochondrial-dysfunction-and-synaptic-damage-in-alzheimer-s-disease
#17
Ramesh Kandimalla, Maria Manczak, David Fry, Yeguvapalli Suneetha, Hiromi Sesaki, P Hemachandra Reddy
The purpose of our study was to understand the protective effects of a partial reduction of dynamin-related protein 1 (Drp1) in Alzheimer's disease (AD) progression and pathogenesis. Increasing evidence suggests that phosphorylated Tau and mitochondrial abnormalities are involved in the loss of synapses, defective axonal transport and cognitive decline, in patients with AD. In the current study, we investigated whether a partial reduction of Drp1 protect neurons from phosphorylated Tau-induced mitochondrial and synaptic toxicities in AD progression...
September 15, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27623243/regulation-of-mitoflash-biogenesis-and-signaling-by-mitochondrial-dynamics
#18
Wenwen Li, Tao Sun, Beibei Liu, Di Wu, Wenfeng Qi, Xianhua Wang, Qi Ma, Heping Cheng
Mitochondria are highly dynamic organelles undergoing constant network reorganization and exhibiting stochastic signaling events in the form of mitochondrial flashes (mitoflashes). Here we investigate whether and how mitochondrial network dynamics regulate mitoflash biogenesis and signaling. We found that mitoflash frequency was largely invariant when network fragmentized or redistributed in the absence of mitofusin (Mfn) 1, Mfn2, or Kif5b. However, Opa1 deficiency decreased spontaneous mitoflash frequency due to superimposing changes in respiratory function, whereas mitoflash response to non-metabolic stimulation was unchanged despite network fragmentation...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27620821/-what-makes-some-rats-live-so-long-the-mitochondrial-contribution-to-longevity-through-balance-of-mitochondrial-dynamics-and-mtdna-content
#19
Anna Picca, Vito Pesce, Giuseppe Sirago, Flavio Fracasso, Christiaan Leeuwenburgh, Angela Maria Serena Lezza
Extremely interesting for aging research are those individuals able to reach older ages still with functions similar to those of younger counterparts. We examined liver samples from ad libitum-fed old (28-month-old, AL-28) and ad libitum-fed very old (32-month-old, AL-32) rats for a number of markers, relevant for mitochondrial functionality and mitochondrial DNA (mtDNA) content. As for the mtDNA content and the protein amounts of the citrate synthase and the antioxidant peroxiredoxin III there were no significant changes in the AL-32 animals...
September 13, 2016: Experimental Gerontology
https://www.readbyqxmd.com/read/27575337/when-mfn2-mitofusin-2-met-autophagy-a-new-age-for-old-muscles
#20
David Sebastián, Antonio Zorzano
A long-standing quest is to define the mechanisms responsible for the mitochondrial dysfunction and accumulation of damaged mitochondria that occur during aging. Indeed, those defects are considered major contributors to the aging process. We have analyzed the effect of aging on the muscle expression of Mfn2 and the impact of Mfn2 ablation on muscle function. Our findings reveal that Mfn2 is repressed in muscle during aging, and that is a determinant for the inhibition of autophagy, and mitochondrial quality control, which lead to the accumulation of damaged mitochondria...
November 2016: Autophagy
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