Read by QxMD icon Read


Alberto Galvez-Ruiz, Alicia Galindo-Ferreiro, Patrik Schatz
In this study, the authors present a sample of 71 patients with hereditary optic neuropathy and negative genetic test results for OPA1/OPA3/LHON. All of these patients later underwent genetic testing to rule out WFS. As a result, 53 patients (74.7%) were negative and 18 patients (25.3%) were positive for some type of mutation or variation in the WFS gene. The authors believe that this study is interesting because it shows that a sizeable percentage (25.3%) of patients with hereditary optic 25 neuropathy and negative genetic test results for OPA1/OPA3/LHON had WFS mutations or variants...
April 2018: Neuro-ophthalmology
Francesco Consolato, Francesca Maltecca, Susanna Tulli, Irene Sambri, Giorgio Casari
The proteolytic processing of dynamin like GTPase OPA1, mediated by the activity of both YME1L1 ( i- AAA protease complex) and OMA1, is a crucial step in the regulation of mitochondrial dynamics. OMA1 is a zinc metallopeptidase of the inner mitochondrial membrane that undergoes pre-activating proteolytic and auto-proteolytic cleavage after mitochondrial import. Here, we identify AFG3L2 ( m- AAA complex) as the major protease mediating this event by maturing the pre-pro-OMA1 of 60 kDa to the pro-OMA1 form of 40 kDa by severing the amino-terminal part without recognizing specific consensus sequence...
March 15, 2018: Journal of Cell Science
Benjamin Gottschalk, Christinae Klec, Markus Waldeck-Weiermair, Roland Malli, Wolfgang F Graier
Mitochondria are multifunctional organelles that essentially contribute to cell signaling by sophisticated mechanisms of communications. Live cell imaging studies showed that mitochondria are dynamic and complex structures that form ramified networks by directed movements, fission, and fusion events. There is emerging evidence that the morphology of mitochondria determines cellular functions and vice versa. Several intracellular signaling pathways and messengers including Ca2+ dynamically influence the architecture of mitochondria...
March 12, 2018: Pflügers Archiv: European Journal of Physiology
Francesca Martorana, Daniela Gaglio, Maria Rosaria Bianco, Federica Aprea, Assunta Virtuoso, Marcella Bonanomi, Lilia Alberghina, Michele Papa, Anna Maria Colangelo
Neuronal differentiation involves extensive modification of biochemical and morphological properties to meet novel functional requirements. Reorganization of the mitochondrial network to match the higher energy demand plays a pivotal role in this process. Mechanisms of neuronal differentiation in response to nerve growth factor (NGF) have been largely characterized in terms of signaling, however, little is known about its impact on mitochondrial remodeling and metabolic function. In this work, we show that NGF-induced differentiation requires the activation of autophagy mediated by Atg9b and Ambra1, as it is disrupted by their genetic knockdown and by autophagy blockers...
March 9, 2018: Cell Death & Disease
Haojuan Jia, Jia Shi, Shu'an Dong, Yuan Zhang, Jianbo Yu
OBJECTIVE: To investigate the effects of heme oxygenase-1/carbon monoxide (HO-1/CO) pathway on mitochondrial fusion in rat alveolar epithelial type II cells (AEC II) stimulated by lipopolysaccharide (LPS). METHODS: Once the cultured in vitro rat AEC II cells line RLE-6TN reached confluency of 85%, they were subcultured and randomly divided into seven groups (n = 5 each). RLE-6TN cells were routinely cultured in control group. The cells in LPS group was stimulated with 10 mg/L LPS to reproduce the model of endotoxin challenge in AECII cells...
March 2018: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
F Garganese, A Ippolito, V di Rienzo, C Lotti, C Montemurro, S M Sanzani
BACKGROUND: Alternaria brown spot is one of the most important diseases of tangerines and their hybrids worldwide. To set up effective control strategy, the accurate detection and identification of the species responsible of the diseases is crucial. However, the characterization based on morphology and/or multilocus genetic approaches is time consuming, requires great expertise, and sometimes is not conclusive. Therefore, the setup of a rapid and efficient DNA-based assay might be of paramount importance...
March 5, 2018: Journal of the Science of Food and Agriculture
Aurel Popa-Wagner, Raluca E Sandu, Coman Cristin, Adriana Uzoni, Kevin A Welle, Jennifer R Hryhorenko, Sina Ghaemmaghami
Brain structures differ in the magnitude of age-related neuron loss with the cerebellum being more affected. An underlying cause could be an age-related decline in mitochondrial bioenergetics. Successful aging of mitochondria reflects a balanced turnover of proteins involved in mitochondrial biogenesis and mitophagy. Thus, an imbalance in mitochondrial turnover can contribute to the diminution of cellular function seen during aging. Mitochondrial biogenesis and mitophagy are mediated by a set of proteins including MFN1, MFN2, OPA1, DRP1, FIS1 as well as DMN1l and DNM1, all of which are required for mitochondrial fission...
2018: Frontiers in Aging Neuroscience
Tianzheng Yu, Iman Ferdjallah, Falicia Elenberg, Star K Chen, Patricia Deuster, Yifan Chen
AIMS: We have previously demonstrated in vitro that heat-induced skeletal muscle damage is associated with an increase in dynamin-related protein 1 (Drp1)-mediated mitochondrial fission and no change in mitochondrial fusion. In this study, we investigated the in vivo effects of mitochondrial fission inhibition on heat-induced oxidative skeletal muscle injury and hyperthermic response in mice. MAIN METHODS: Core body temperatures of mice pre-treated with vehicle or Mdivi-1 were recorded by radio telemetry during heat exposure...
February 27, 2018: Life Sciences
Yongzheng Guo, Zhen Wang, Xinghua Qin, Jie Xu, Zuoxu Hou, Hongyan Yang, Xuechao Mao, Wenjuan Xing, Xiaoliang Li, Xing Zhang, Feng Gao
Aims: Heart failure is characterized by reduced fatty acid (FA) utilization associated with mitochondrial dysfunction. Recent evidence has shown that enhancing FA utilization may provide cardioprotection against heart failure. Our aim was to investigate the effects and the underlying mechanisms of cardiac FA utilization on cardiac function in response to pressure overload. Methods and Results: Transverse aortic constriction (TAC) was used in C57 mice to establish pressure overload-induced heart failure...
February 27, 2018: Cardiovascular Research
Angelo Iannielli, Simone Bido, Lucrezia Folladori, Alice Segnali, Cinzia Cancellieri, Alessandra Maresca, Luca Massimino, Alicia Rubio, Giuseppe Morabito, Leonardo Caporali, Francesca Tagliavini, Olimpia Musumeci, Giuliana Gregato, Erwan Bezard, Valerio Carelli, Valeria Tiranti, Vania Broccoli
Dysfunctions in mitochondrial dynamics and metabolism are common pathological processes associated with Parkinson's disease (PD). It was recently shown that an inherited form of PD and dementia is caused by mutations in the OPA1 gene, which encodes for a key player in mitochondrial fusion and structure. iPSC-derived neural cells from these patients exhibited severe mitochondrial fragmentation, respiration impairment, ATP deficits, and heightened oxidative stress. Reconstitution of normal levels of OPA1 in PD-derived neural cells normalized mitochondria morphology and function...
February 20, 2018: Cell Reports
Jessica Sacks, Anny Mulya, Ciaran E Fealy, Hazel Huang, John D Mosinski, Mangesh R Pagadala, Hideharu Shimizu, Esam Batayyah, Philip R Schauer, Stacy A Brethauer, John P Kirwan
Bariatric surgery provides significant and durable improvements in glycemic control and hepatic steatosis, but the underlying mechanisms that drive improvements in these metabolic parameters remain to be fully elucidated. Recently, alterations in mitochondrial morphology have shown a direct link to nutrient adaptations in obesity. Here, we evaluate the effects of Roux-en-Y gastric bypass (RYGB) surgery on markers of liver mitochondrial dynamics in a diet-induced obesity Sprague-Dawley (SD) rat model. Livers were harvested from adult male SD rats 90-days after either Sham or RYGB surgery and continuous high-fat feeding...
February 2018: Physiological Reports
Jonathan Ausman, Joelcio Abbade, Leonardo Ermini, Abby Farrell, Andrea Tagliaferro, Martin Post, Isabella Caniggia
Mitochondria are in a constant balance of fusing and dividing in response to cellular cues. Fusion creates healthy mitochondria, whereas fission results in removal of non-functional organelles. Changes in mitochondrial dynamics typify several human diseases. However, the contribution of mitochondrial dynamics to preeclampsia, a hypertensive disorder of pregnancy characterized by placental cell autophagy and death, remains unknown. Herein, we show that the mitochondrial dynamic balance in preeclamptic placentae is tilted toward fission (increased DRP1 expression/activation and decreased OPA1 expression)...
February 20, 2018: Cell Death & Disease
Philip Böhler, Fabian Stuhldreier, Ruchika Anand, Arun Kumar Kondadi, David Schlütermann, Niklas Berleth, Jana Deitersen, Nora Wallot-Hieke, Wenxian Wu, Marian Frank, Hendrik Niemann, Elisabeth Wesbuer, Andreas Barbian, Tomas Luyten, Jan B Parys, Stefanie Weidtkamp-Peters, Andrea Borchardt, Andreas S Reichert, Aida Peña-Blanco, Ana J García-Sáez, Samuel Itskanov, Alexander M van der Bliek, Peter Proksch, Sebastian Wesselborg, Björn Stork
Mitochondria are cellular organelles with crucial functions in the generation and distribution of ATP, the buffering of cytosolic Ca2+ and the initiation of apoptosis. Compounds that interfere with these functions are termed mitochondrial toxins, many of which are derived from microbes, such as antimycin A, oligomycin A, and ionomycin. Here, we identify the mycotoxin phomoxanthone A (PXA), derived from the endophytic fungus Phomopsis longicolla, as a mitochondrial toxin. We show that PXA elicits a strong release of Ca2+ from the mitochondria but not from the ER...
February 19, 2018: Cell Death & Disease
Valentina Del Dotto, Mario Fogazza, Guy Lenaers, Michela Rugolo, Valerio Carelli, Claudia Zanna
OPA1 is a GTPase that controls several functions, such as mitochondrial dynamics and energetics, mtDNA maintenance and cristae integrity. In the last years, there have been described other cellular pathways and mechanisms involving OPA1 directly or through its interaction. All this new information, by implementing our knowledge on OPA1 is instrumental to elucidating the pathogenic mechanisms of OPA1 mutations. Indeed, these are associated with dominant optic atrophy (DOA), one of the most common inherited optic neuropathies, and with an increasing number of heterogeneous neurodegenerative disorders...
February 15, 2018: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
Megan L Rasmussen, Leigh A Kline, Kyungho P Park, Natalya A Ortolano, Alejandra I Romero-Morales, Christin C Anthony, Kathryn E Beckermann, Vivian Gama
Human pluripotent stem cells (hPSCs) maintain a highly fragmented mitochondrial network, but the mechanisms regulating this phenotype remain unknown. Here, we describe a non-cell death function of the anti-apoptotic protein, MCL-1, in regulating mitochondrial dynamics and promoting pluripotency of stem cells. MCL-1 is induced upon reprogramming, and its inhibition or knockdown induces dramatic changes to the mitochondrial network as well as loss of the key pluripotency transcription factors, NANOG and OCT4...
January 31, 2018: Stem Cell Reports
Emmanuelle Sarzi, Marie Seveno, Camille Piro-Mégy, Lucie Elzière, Mélanie Quilès, Marie Péquignot, Agnès Müller, Christian P Hamel, Guy Lenaers, Cécile Delettre
Dominant optic atrophy (DOA) is a rare progressive and irreversible blinding disease which is one of the most frequent forms of hereditary optic neuropathy. DOA is mainly caused by dominant mutation in the OPA1 gene encoding a large mitochondrial GTPase with crucial roles in membrane dynamics and cell survival. Hereditary optic neuropathies are commonly characterized by the degeneration of retinal ganglion cells, leading to the optic nerve atrophy and the progressive loss of visual acuity. Up to now, despite increasing advances in the understanding of the pathological mechanisms, DOA remains intractable...
February 6, 2018: Scientific Reports
Maria Manczak, Ramesh Kandimalla, Xiangling Yin, P Hemachandra Reddy
The purpose of our study was to determine the toxic effects of hippocampal mutant APP and amyloid beta (Aβ) in 12-month-old APP transgenic mice. Using rotarod and Morris Water Maze tests, immunoblotting & immunofluorescence, Golgi-cox staining and transmission electron microscopy, we assessed cognitive behavior, protein levels of synaptic, autophagy, mitophagy, mitochondrial dynamics, biogenesis, dendritic protein MAP2, and quantified dendritic spines and mitochondrial number and length in 12-month-old APP mice that express swedish mutation...
February 1, 2018: Human Molecular Genetics
Songyun Deng, Yuhang Ai, Hua Gong, Qing Feng, Xiao Li, Caixia Chen, Zhiyong Liu, Yimin Wang, Qianyi Peng, Lina Zhang
Sepsis is one of the most common reasons for mortality in Intensive Care Units. As a common but severe neurological complication, sepsis associated encephalopathy (SAE) has always been ignored and there is no generally accepted treatment. In this study, we demonstrated that Mdivi-1 ameliorated brain damage assessed by Nissl staining. Furthermore, Mdivi-1 reduced TUNEL-positive cells in hippocampus, and inhibited S100 calcium binding protein B (S100B) and neuron-specific enolase (NSE) release into plasma. Biochemical analysis also showed that Mdivi-1 protected hippocampus from oxidative stresses...
January 26, 2018: Biochemical and Biophysical Research Communications
Valentina Del Dotto, Mario Fogazza, Valerio Carelli, Michela Rugolo, Claudia Zanna
OPA1 is a dynamin-related GTPase that controls mitochondrial dynamics, cristae integrity, energetics and mtDNA maintenance. The exceptional complexity of this protein is determined by the presence, in humans, of eight different isoforms that, in turn, are proteolytically cleaved into combinations of membrane-anchored long forms and soluble short forms. Recent advances highlight how each OPA1 isoform is able to fulfill "essential" mitochondrial functions, whereas only some variants carry out "specialized" features...
January 27, 2018: Biochimica et Biophysica Acta
Aliz Szabo, Katalin Sumegi, Katalin Fekete, Eniko Hocsak, Balazs Debreceni, Gyorgy Setalo, Krisztina Kovacs, Laszlo Deres, Andras Kengyel, Dominika Kovacs, Jozsef Mandl, Miklos Nyitrai, Mark A Febbraio, Ferenc Gallyas, Balazs Sumegi
Mitochondria fragmentation destabilizes mitochondrial membranes, promotes oxidative stress and facilitates cell death, thereby contributing to the development and the progression of several mitochondria-related diseases. Accordingly, compounds that reverse mitochondrial fragmentation could have therapeutic potential in treating such diseases. BGP-15, a hydroxylamine derivative, prevents insulin resistance in humans and protects against several oxidative stress-related diseases in animal models. Here we show that BGP-15 promotes mitochondrial fusion by activating optic atrophy 1 (OPA1), a GTPase dynamin protein that assist fusion of the inner mitochondrial membranes...
January 26, 2018: Biochemical Pharmacology
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"