keyword
MENU ▼
Read by QxMD icon Read
search

Mitochondrial disease

keyword
https://www.readbyqxmd.com/read/29235274/-identification-and-function-analysis-of-target-group-for-cardioprotection-of-baoyuan-decoction
#1
Yan-Jun Wan, Li-Xi Liao, Yu-Qi Liu, Yong Jiang, Lian-Ying Liu, Ke-Wu Zeng, Peng-Fei Tu
Baoyuan decoction (BYD) is a well-known traditional Chinese medicine formula for coronary heart disease with Qi deficiency. However, the detailed pharmacological mechanism of BYD is still unknown because of its complicated chemical compositions. In this study, we synthesized a kind of solid beads with benzophenone groups on its surface. Benzophenone can be activated and chemically cross-linked with the C-H bonds of the chemical compositions in BYD (BYD beads) under UV activation. We thus captured all the target proteins from mouse heart tissue lysates by using BYD beads...
October 2017: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
https://www.readbyqxmd.com/read/29235175/acetate-provokes-mitochondrial-stress-and-cell-death-in-ustilago-maydis
#2
Matthias Kretschmer, Scott Lambie, Daniel Croll, James W Kronstad
The fungal pathogen Ustilago maydis causes disease on maize by mating to establish an infectious filamentous cell type that invades the host and induces tumours. We previously found that β-oxidation mutants were defective in virulence and did not grow on acetate. Here we demonstrate that acetate inhibits filamentation during mating and in response to oleic acid. We therefore examined the influence of different carbon sources by comparing the transcriptomes of cells grown on acetate, oleic acid or glucose, with expression changes for the fungus during tumour formation in planta...
December 13, 2017: Molecular Microbiology
https://www.readbyqxmd.com/read/29235132/mitochondrial-donation-patient-engagement-and-narratives-of-hope
#3
Cathy Herbrand, Rebecca Dimond
This article develops the sociology of hope and patient engagement by exploring how patients' perceptions and actions are shaped by narratives of hope surrounding the clinical introduction of novel reproductive techniques. In 2015, after extensive public debates, the UK became the first country to legalise a mitochondrial donation technique aimed at preventing the transmission of inherited disorders. The article draws on the accounts of twenty-two women of reproductive age who are at risk of having a child with mitochondrial disease and would be the potential target of the technique...
December 12, 2017: Sociology of Health & Illness
https://www.readbyqxmd.com/read/29235020/do-gstm1-and-gstt1-polymorphisms-influence-the-risk-of-developing-mitochondrial-diseases-in-a-tunisian-population
#4
Raouia Ghorbel, Ghada Ben Salah, Rania Ghorbel, Afif Ben Mahmoud, Imen Chamkha, Emna Mkaouar-Rebai, Leila Ammar-Keskes, Faiza Fakhfakh
Mitochondria play an essential role to supply the cell with metabolic energy in the form of adenosine triphosphate (ATP) through oxidative phosphorylation (OXPHOS). As a consequence, they are also the primary source of cellular reactive oxygen species (ROS) which can cause oxidative damage of individual respiratory chain complexes. Indeed, affected OXPHOS subunits result in decreases in ATP production and increases in ROS formation which generate oxidative phosphorylation deficiency leading to mitochondrial dysfunctions...
December 12, 2017: Environmental Science and Pollution Research International
https://www.readbyqxmd.com/read/29234478/fructose-ingestion-impairs-expression-of-genes-involved-in-skeletal-muscle-s-adaptive-response-to-aerobic-exercise
#5
Natalia Gomes Gonçalves, Stephanie Heffer Cavaletti, Carlos Augusto Pasqualucci, Milton Arruda Martins, Chin Jia Lin
Background: The inverse relationship between exercise capacity and its variation over time and both cardiovascular and all-cause mortality suggests the existence of an etiological nexus between cardiometabolic diseases and the molecular regulators of exercise capacity. Coordinated adaptive responses elicited by physical training enhance exercise performance and metabolic efficiency and possibly mediate the health benefits of physical exercise. In contrast, impaired expression of genes involved in mitochondrial biogenesis or protein turnover in skeletal muscle-key biological processes involved in adaptation to physical training-leads to insulin resistance and obesity...
2017: Genes & Nutrition
https://www.readbyqxmd.com/read/29234200/nutrition-and-muscle-in-cirrhosis
#6
REVIEW
Anil C Anand
As the cirrhosis progresses, development of complication like ascites, hepatic encephalopathy, variceal bleeding, kidney dysfunction, and hepatocellular carcinoma signify increasing risk of short term mortality. Malnutrition and muscle wasting (sarcopenia) is yet other complications that negatively impact survival, quality of life, and response to stressors, such as infection and surgery in patients with cirrhosis. Conventionally, these are not routinely looked for, because nutritional assessment can be a difficult especially if there is associated fluid retention and/or obesity...
December 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29234155/analysis-of-neuronal-phosphoproteome-reveals-pink1-regulation-of-bad-function-and-cell-death
#7
Huida Wan, Bin Tang, Xun Liao, Qiufang Zeng, Zhuohua Zhang, Lujian Liao
PINK1 mutations that disrupt its kinase activity cause autosomal recessive early onset Parkinson's disease (PD). Although research in recent years has elucidated a PINK1-Parkin pathway of mitophagy activation that requires PINK1 kinase activity, mitophagy-independent functions of PINK1 and their possible roles in PD pathogenesis have been proposed. Using an unbiased quantitative mass spectrometry approach to analyze the phosphoproteome in primary neurons from wild type and Pink1 knockout mice after mitochondrial depolarization, we uncovered PINK1-regulated phosphorylation sites, which involve coordinated activation of multiple signaling pathways that control cellular response to stress...
December 12, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29234100/a-bi-fluorescence-complementation-system-to-detect-associations-between-the-endoplasmic-reticulum-and-mitochondria
#8
Mark Harmon, Philip Larkman, Giles Hardingham, Mandy Jackson, Paul Skehel
Close contacts between the endoplasmic reticulum membrane and the mitochondrial outer membrane facilitate efficient transfer of lipids between the organelles and coordinate Ca2+ signalling and stress responses. Changes to this coupling is associated with a number of metabolic disorders and neurodegenerative diseases including Alzheimer's, Parkinson's and motor neuron disease. The distance between the two membranes at regions of close apposition is below the resolution of conventional light microscopy, which makes analysis of these interactions challenging...
December 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29234042/cfdna-correlates-with-endothelial-damage-after-cardiac-surgery-with-prolonged-cardiopulmonary-bypass-and-amplifies-netosis-in-an-intracellular-tlr9-independent-manner
#9
Adnana Paunel-Görgülü, Max Wacker, Mouhamed El Aita, Shoreshfan Hassan, Georg Schlachtenberger, Antje Deppe, Yeong-Hoon Choi, Elmar Kuhn, Thorsten O Mehler, Thorsten Wahlers
Cardiopulmonary bypass (CPB) provokes inflammation culminating in organ dysfunction and increased mortality. Recently, neutrophil extracellular traps (NETs) have been found to be involved in a variety of cardiovascular diseases promoting tissue and organ injury. Here, we aimed to elaborate the proinflammatory potential of circulating cell-free (cf)DNA in patients undergoing cardiac surgery with CPB. Plasma was collected pre- and postoperatively as well as at d1, d3, d5 and d8 after surgery. At d1, we found circulating cfDNA levels to be significantly increased in patients with prolonged CPB duration (>100 min) when compared to those with shorter CPB times (CPB < 100 min)...
December 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29233996/pepd-is-a-pivotal-regulator-of-p53-tumor-suppressor
#10
Lu Yang, Yun Li, Arup Bhattacharya, Yuesheng Zhang
p53 tumor suppressor responds to various cellular stresses and regulates cell fate. Here, we show that peptidase D (PEPD) binds and suppresses over half of nuclear and cytoplasmic p53 under normal conditions, independent of its enzymatic activity. Eliminating PEPD causes cell death and tumor regression due to p53 activation. PEPD binds to the proline-rich domain in p53, which inhibits phosphorylation of nuclear p53 and MDM2-mediated mitochondrial translocation of nuclear and cytoplasmic p53. However, the PEPD-p53 complex is critical for p53 response to stress, as stress signals doxorubicin and H2O2 each must free p53 from PEPD in order to achieve robust p53 activation, which is mediated by reactive oxygen species...
December 12, 2017: Nature Communications
https://www.readbyqxmd.com/read/29233888/mitochondrial-energy-generation-disorders-genes-mechanisms-and-clues-to-pathology
#11
Ann E Frazier, David R Thorburn, Alison G Compton
Inherited disorders of oxidative phosphorylation cause the clinically and genetically heterogeneous diseases known as mitochondrial energy generation disorders, or mitochondrial diseases. Over the last three decades, mutations causing these disorders have been identified in almost 290 genes, but many patients still remain without a molecular diagnosis. Moreover, while knowledge of the genetic causes is continually expanding, understanding into how these defects lead to cellular dysfunction and organ pathology is still incomplete...
December 12, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29233845/physiologic-mitochondrial-fragmentation-is-a-normal-cardiac-adaptation-to-increased-energy-demand
#12
Michael Coronado, Giovanni Fajardo, Kim Nguyen, Mingming Zhao, Kristina B Kooiker, Gwanghyun Jung, Dong-Qing Hu, Sushma Reddy, Erik Sandoval, Aleksandr Stotland, Roberta A Gottlieb, Daniel Bernstein
Rationale: Mitochondria play a dual role in the heart, responsible for meeting energetic demands and regulating cell death. Paradigms have held that mitochondrial fission and fragmentation are the result of pathologic stresses such as ischemia, are an indicator of poor mitochondrial health, and lead to mitophagy and cell death. However, recent studies demonstrate that inhibiting fission also results in decreased mitochondrial function and cardiac impairment, suggesting that fission is important for maintaining cardiac and mitochondrial bioenergetic homeostasis...
December 12, 2017: Circulation Research
https://www.readbyqxmd.com/read/29233065/understanding-the-role-of-glycogen-synthase-kinase-3-in-l-dopa-induced-dyskinesia-in-parkinson-s-disease
#13
Hojin Choi, Seong-Ho Koh
Levodopa (L-DOPA) is the most commonly used drug for Parkinson's disease (PD), but its long-term use is associated with various complications, including L-DOPA-induced dyskinesia (LID). Many studies have suggested that L-DOPA neurotoxicity and LID are associated with glycogen synthase kinase-3 (GSK-3) activation. Areas covered: LID is caused by striatal dopamine (DA) denervation in PD and pulsatile L-DOPA treatment. These factors lead to dysregulated DA transmission, abnormal intracellular signaling and transcription factors in striatal neurons, and altered gene expression and plasticity at corticostriatal synapses...
December 12, 2017: Expert Opinion on Drug Metabolism & Toxicology
https://www.readbyqxmd.com/read/29232010/protective-effect-of-hsp27-in-atherosclerosis-and-coronary-heart-disease-by-inhibiting-reactive-oxygen-species
#14
Hongli Zhang, Kaiying Jia, Da Sun, Min Yang
OBJECTIVE: To clarify the mechanism of HSP27 as a diagnostic biomarker in coronary heart disease (CHD) and atherosclerosis (AS). METHOD: Expressions of HSP27 in CHD patients and healthy controls were determined by ELISA, and expressions of HSP27 in aortas of CHD patients and healthy controls were measured by Immunohistochemistry. ROC curve was applied to assess the diagnostic performance of HSP27 in CHD. ApoE-/- mice were included and accordingly grouped. The expressions of HSP27 in AS plaque were measured by qRT-PCR, Immunohistochemistry and Western blot...
December 12, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29229998/guidelines-on-experimental-methods-to-assess-mitochondrial-dysfunction-in-cellular-models-of-neurodegenerative-diseases
#15
REVIEW
Niamh M C Connolly, Pierre Theurey, Vera Adam-Vizi, Nicolas G Bazan, Paolo Bernardi, Juan P Bolaños, Carsten Culmsee, Valina L Dawson, Mohanish Deshmukh, Michael R Duchen, Heiko Düssmann, Gary Fiskum, Maria F Galindo, Giles E Hardingham, J Marie Hardwick, Mika B Jekabsons, Elizabeth A Jonas, Joaquin Jordán, Stuart A Lipton, Giovanni Manfredi, Mark P Mattson, BethAnn McLaughlin, Axel Methner, Anne N Murphy, Michael P Murphy, David G Nicholls, Brian M Polster, Tullio Pozzan, Rosario Rizzuto, Jorgina Satrústegui, Ruth S Slack, Raymond A Swanson, Russell H Swerdlow, Yvonne Will, Zheng Ying, Alvin Joselin, Anna Gioran, Catarina Moreira Pinho, Orla Watters, Manuela Salvucci, Irene Llorente-Folch, David S Park, Daniele Bano, Maria Ankarcrona, Paola Pizzo, Jochen H M Prehn
Neurodegenerative diseases are a spectrum of chronic, debilitating disorders characterised by the progressive degeneration and death of neurons. Mitochondrial dysfunction has been implicated in most neurodegenerative diseases, but in many instances it is unclear whether such dysfunction is a cause or an effect of the underlying pathology, and whether it represents a viable therapeutic target. It is therefore imperative to utilise and optimise cellular models and experimental techniques appropriate to determine the contribution of mitochondrial dysfunction to neurodegenerative disease phenotypes...
December 11, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29229602/vegfr-2-mediated-reprogramming-of-mitochondrial-metabolism-regulates-the-sensitivity-of-acute-myeloid-leukemia-to-chemotherapy
#16
Sandrina Nóbrega-Pereira, Francisco Caiado, Tânia Carvalho, Inês Matias, Gonçalo Graça, Luís Gafeira Gonçalves, Bruno Silva-Santos, Haakan Norell, Sérgio Dias
Metabolic reprogramming is central to tumorigenesis, but whether chemotherapy induces metabolic features promoting recurrence remains unknown. We established a mouse xenograft model of human acute myeloid leukemia (AML) that enabled chemotherapy-induced regressions of established disease followed by lethal regrowth of more aggressive tumor cells. Human AML cells from terminally ill mice treated with chemotherapy (chemoAML) had higher lipid content, increased lactate production and ATP levels, reduced expression of PPARG coactivator 1α (PGC-1α), and fewer mitochondria than controls from untreated AML animals...
December 11, 2017: Cancer Research
https://www.readbyqxmd.com/read/29228971/alpha-synuclein-oligomer-selective-antibodies-reduce-intracellular-accumulation-and-mitochondrial-impairment-in-alpha-synuclein-exposed-astrocytes
#17
Gabriel Gustafsson, Veronica Lindström, Jinar Rostami, Eva Nordström, Lars Lannfelt, Joakim Bergström, Martin Ingelsson, Anna Erlandsson
BACKGROUND: Due to its neurotoxic properties, oligomeric alpha-synuclein (α-syn) has been suggested as an attractive target for passive immunization against Parkinson's disease (PD). In mouse models of PD, antibody treatment has been shown to lower the levels of pathogenic α-syn species, including oligomers, although the mechanisms of action remain unknown. We have previously shown that astrocytes rapidly engulf α-syn oligomers that are intracellularly stored, rather than degraded, resulting in impaired mitochondria...
December 11, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29228836/complete-mtdna-sequencing-reveals-mutations-m-9185t-c-and-m-13513g-a-in-three-patients-with-leigh-syndrome
#18
Dita Pelnena, Birute Burnyte, Eriks Jankevics, Baiba Lace, Evelina Dagyte, Kristina Grigalioniene, Algirdas Utkus, Zita Krumina, Jolanta Rozentale, Irina Adomaitiene, Janis Stavusis, Liana Pliss, Inna Inashkina
The most common mitochondrial disorder in children is Leigh syndrome, which is a progressive and genetically heterogeneous neurodegenerative disorder caused by mutations in nuclear genes or mitochondrial DNA (mtDNA). In the present study, a novel and robust method of complete mtDNA sequencing, which allows amplification of the whole mitochondrial genome, was tested. Complete mtDNA sequencing was performed in a cohort of patients with suspected mitochondrial mutations. Patients from Latvia and Lithuania (n = 92 and n = 57, respectively) referred by clinical geneticists were included...
December 12, 2017: Mitochondrial DNA. Part A. DNA Mapping, Sequencing, and Analysis
https://www.readbyqxmd.com/read/29227178/neuronal-pathophysiology-featuring-prpc-and-its-control-over-ca2-metabolism
#19
Alessandro Bertoli, M Catia Sorgato
Calcium (Ca2+) is an intracellular second messenger that ubiquitously masters remarkably diverse biological processes, including cell death. Growing evidence substantiates an involvement of the prion protein (PrPC) in regulating neuronal Ca2+ homeostasis, which could rationalize most of the wide range of functions ascribed to the protein. We have recently demonstrated that PrPC controls extracellular Ca2+ fluxes, and mitochondrial Ca2+ uptake, in neurons stimulated with glutamate (De Mario et al., J Cell Sci 2017; 130:2736-46), suggesting that PrPC protects neurons from threatening Ca2+ overloads and excitotoxicity...
December 11, 2017: Prion
https://www.readbyqxmd.com/read/29226533/the-anthelmintic-drug-niclosamide-and-its-analogues-activate-the-parkinson-s-disease-associated-protein-kinase-pink1
#20
Erica Barini, Ageo Miccoli, Federico Tinarelli, Katie Mulholand, Hachemi Kadri, Farhat Khanim, Laste Stojanovski, Kevin D Read, Kerry Burness, Julian J Blow, Youcef Mehellou, Miratul Muqit
Mutations in PINK1, which impair its catalytic kinase activity, are causal for autosomal recessive early onset Parkinson's disease (PD). Various studies have indicated that the activation of PINK1 could be a useful strategy in treating neurodegenerative diseases such as PD. Herein, we show that the anthelmintic drug niclosamide and its analogues are capable of activating PINK1 in cells via reversible impairment of the mitochondrial membrane potential. Using these compounds, we demonstrate for the first time that the PINK1 pathway is active and detectable in primary neurons...
December 10, 2017: Chembiochem: a European Journal of Chemical Biology
keyword
keyword
20488
1
2
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"