keyword
MENU ▼
Read by QxMD icon Read
search

Mitochondrial diseases

keyword
https://www.readbyqxmd.com/read/28448943/mitochondria-in-endothelial-cells-sensors-and-integrators-of-environmental-cues
#1
REVIEW
Sergio Caja, Jose Antonio Enríquez
The involvement of angiogenesis in disease and its potential as a therapeutic target have been firmly established over recent decades. Endothelial cells (ECs) are central elements in vessel homeostasis and regulate the passage of material and cells into and out of the bloodstream. EC proliferation and migration are modified by alterations to mitochondrial biogenesis and dynamics resulting from several signals and environmental cues, such as oxygen, hemodynamics, and nutrients. As intermediary signals, mitochondrial ROS are released as important downstream modulators of the expression of angiogenesis-related genes...
April 18, 2017: Redox Biology
https://www.readbyqxmd.com/read/28448247/oleuropein-isolated-from-fraxinus-rhynchophylla-inhibits-glutamate-induced-neuronal-cell-death-by-attenuating-mitochondrial-dysfunction
#2
Mi Hye Kim, Ju-Sik Min, Joon Yeop Lee, Unbin Chae, Eun-Ju Yang, Kyung-Sik Song, Hyun-Shik Lee, Hong Jun Lee, Sang-Rae Lee, Dong-Seok Lee
Glutamate-induced neurotoxicity is related to excessive oxidative stress accumulation and results in the increase of neuronal cell death. In addition, glutamate has been reported to lead to neurodegenerative diseases, including Parkinson's and Alzheimer's diseases.It is well known that Fraxinus rhynchophylla contains a significant level of oleuropein (Ole), which exerts various pharmacological effects. However, the mechanism of neuroprotective effects of Ole is still poorly defined. In this study, we aimed to investigate whether Ole prevents glutamate-induced toxicity in HT-22 hippocampal neuronal cells...
April 27, 2017: Nutritional Neuroscience
https://www.readbyqxmd.com/read/28447797/signal-transductions-of-beas-2b-cells-in-response-to-carcinogenic-pm2-5-exposure-based-on-microfluidic-system
#3
Lulu Zheng, Sixiu Liu, Guoshun Zhuang, Jian Xu, Qi Liu, Xinlian Zhang, Congrui Deng, Zhigang Guo, Wang Zhao, Tingna Liu, Yiqi Wang, Yuxiao Zhang, Jing Lin, Qiongzhen Wang, Guodong Sui
PM2.5 (particulate matter less than 2.5 micrometers in diameter) is considered as a harmful carcinogen. Determining the precise relationship between the chemical constituents of PM2.5 in the air and cancer progression could aid the treatment of environment related disease and establishing risk reduction strategies. Herein, we used transcriptomics (RNA-seq) and integrated microfluidic system to identify the global gene expression and differential target proteins expression induced by ambient fine particles collected from the heavy haze in China...
April 27, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/28447714/resveratrol-protects-against-oxidized-low%C3%A2-density-lipoprotein%C3%A2-induced-human-umbilical-vein-endothelial-cell-apoptosis-via-inhibition-of-mitochondrial%C3%A2-derived-oxidative-stress
#4
Yujie Liu, Xizhou Chen, Jie Li
Resveratrol, a natural phytochemical found in grapes and red wine, has been found to possess protective effects against endothelial cell apoptosis and oxidative damage. Oxidized‑low density lipoprotein (ox‑LDL) can induce apoptosis of endothelial cells, which is an important initial event in several cardiovascular diseases. However, the effect of resveratrol on ox‑LDL‑induced apoptosis and oxidative damage, and the possible associated mechanisms remain to be elucidated. In the present study, following exposure to ox‑LDL, human umbilical vein endothelial cells (HUVECs) were treated with or without resveratrol...
March 9, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28446709/control-of-mitochondrial-biogenesis-and-function-by-the-ubiquitin-proteasome-system
#5
REVIEW
Piotr Bragoszewski, Michal Turek, Agnieszka Chacinska
Mitochondria are pivotal organelles in eukaryotic cells. The complex proteome of mitochondria comprises proteins that are encoded by nuclear and mitochondrial genomes. The biogenesis of mitochondrial proteins requires their transport in an unfolded state with a high risk of misfolding. The mislocalization of mitochondrial proteins is deleterious to the cell. The electron transport chain in mitochondria is a source of reactive oxygen species that damage proteins. Mitochondrial dysfunction is linked to many pathological conditions and, together with the loss of cellular protein homeostasis (proteostasis), are hallmarks of ageing and ageing-related degeneration diseases...
April 2017: Open Biology
https://www.readbyqxmd.com/read/28446676/grass-carp-reovirus-vp41-targets-fish-mita-to-abrogate-the-ifn-response
#6
Long-Feng Lu, Shun Li, Zhao-Xi Wang, Si-Qi Du, Dan-Dan Chen, Pin Nie, Yong-An Zhang
Although fish possess an efficient interferon (IFN) system to defend against aquatic virus infection, the grass carp reovirus (GCRV) still causes hemorrhagic disease in grass carp. To date, GCRV's strategy for evading the fish IFN response is still unknown. Here, we report that the GCRV VP41 inhibits fish IFN production by suppressing the phosphorylation of the mediator of IRF3 activation (MITA). First, the activation of the IFN promoter (IFNpro) stimulated by the mitochondrial antiviral signaling protein (MAVS) and MITA was decreased by the overexpression of VP41, whereas such activation induced by TANK-binding kinase 1 (TBK1) was not affected...
April 26, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28446627/silicon-alleviates-nonalcoholic-steatohepatitis-by-reducing-apoptosis-in-aged-wistar-rats-fed-a-high-saturated-fat-high-cholesterol-diet
#7
Alba Garcimartín, M Elvira López-Oliva, Jorge A Sántos-López, Rosa A García-Fernández, Adrián Macho-González, Sara Bastida, Juana Benedí, Francisco J Sánchez-Muniz
Background: Lipoapoptosis has been identified as a key event in the progression of nonalcoholic fatty liver disease (NAFLD), and hence, antiapoptotic agents have been recommended as a possible effective treatment for nonalcoholic steatohepatitis (NASH). Silicon, included in meat as a functional ingredient, improves lipoprotein profiles and liver antioxidant defenses in aged rats fed a high-saturated fat, high-cholesterol diet (HSHCD). However, to our knowledge, the antiapoptotic effect of this potential functional meat on the liver has never been tested...
April 26, 2017: Journal of Nutrition
https://www.readbyqxmd.com/read/28446534/proteomics-analysis-of-tissue-samples-reveals-changes-in-mitochondrial-protein-levels-in-parathyroid-hyperplasia-over-adenoma
#8
Gurler Akpinar, Murat Kasap, Nuh Zafer Canturk, Mehin Zulfigarova, Eylül Ece Islek, Sertac Ata Guler, Turgay Simsek, Zeynep Canturk
BACKGROUND/AIM: To unveil the pathophysiology of primary hyperparathyroidism, molecular details of parathyroid hyperplasia and adenoma have to be revealed. Such details will provide the tools necessary for differentiation of these two look-alike diseases. Therefore, in the present study, a comparative proteomic study using postoperative tissue samples from the parathyroid adenoma and parathyroid hyperplasia patients was performed. MATERIALS AND METHODS: Protein extracts were prepared from tissue samples (n=8 per group)...
May 2017: Cancer Genomics & Proteomics
https://www.readbyqxmd.com/read/28445716/trumping-neurodegeneration-targeting-common-pathways-regulated-by-autosomal-recessive-parkinson-s-disease-genes
#9
REVIEW
Laura Scott, Valina L Dawson, Ted M Dawson
Parkinson's disease (PD) is a neurodegenerative movement disorder characterized by the progressive loss of dopaminergic (DA) neurons. Most PD cases are sporadic; however, rare familial forms have been identified. Autosomal recessive PD (ARPD) results from mutations in Parkin, PINK1, DJ-1, and ATP13A2, while rare, atypical juvenile ARPD result from mutations in FBXO7, DNAJC6, SYNJ1, and PLA2G6. Studying these genes and their function has revealed mitochondrial quality control, protein degradation processes, and oxidative stress responses as common pathways underlying PD pathogenesis...
April 23, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28445457/the-mitochondrial-na-ca-2-exchanger-is-essential-for-ca-2-homeostasis-and-viability
#10
Timothy S Luongo, Jonathan P Lambert, Polina Gross, Mary Nwokedi, Alyssa A Lombardi, Santhanam Shanmughapriya, April C Carpenter, Devin Kolmetzky, Erhe Gao, Jop H van Berlo, Emily J Tsai, Jeffery D Molkentin, Xiongwen Chen, Muniswamy Madesh, Steven R Houser, John W Elrod
Mitochondrial calcium (mCa(2+)) has a central role in both metabolic regulation and cell death signalling, however its role in homeostatic function and disease is controversial. Slc8b1 encodes the mitochondrial Na(+)/Ca(2+) exchanger (NCLX), which is proposed to be the primary mechanism for mCa(2+) extrusion in excitable cells. Here we show that tamoxifen-induced deletion of Slc8b1 in adult mouse hearts causes sudden death, with less than 13% of affected mice surviving after 14 days. Lethality correlated with severe myocardial dysfunction and fulminant heart failure...
April 26, 2017: Nature
https://www.readbyqxmd.com/read/28444372/quantifying-the-number-of-independent-organelle-dna-insertions-in-genome-evolution-and-human-health
#11
Einat Hazkani-Covo, William F Martin
Fragments of organelle genomes are often found as insertions in nuclear DNA. These fragments of mitochondrial DNA (numts) and plastid DNA (nupts) are ubiquitous components of eukaryotic genomes. They are, however, often edited out during the genome assembly process, leading to systematic underestimation of their frequency. Numts and nupts, once inserted, can become further fragmented through subsequent insertion of mobile elements or other recombinational events that disrupt the continuity of the inserted sequence relative to the genuine organelle DNA copy...
April 21, 2017: Genome Biology and Evolution
https://www.readbyqxmd.com/read/28444186/frataxin-deficiency-impairs-mitochondrial-biogenesis-in-cells-mice-and-humans
#12
Mittal J Jasoliya, Marissa Z McMackin, Chelsea K Henderson, Susan L Perlman, Gino A Cortopassi
Friedreich's ataxia (FRDA) is a neurodegenerative disease caused by inherited deficiency of the mitochondrial protein Frataxin (FXN), which has no approved therapy and is an area in which biomarkers are needed for clinical development. Here we investigated the consequences of FXN deficiency in patient derived FRDA fibroblast cell models, the FRDA mouse model KIKO, and in whole blood collected from FRDA patients. We observed decreased mitochondrial copy number in all the three FRDA models tested: cells, mice and patient blood...
April 21, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28443623/novel-slc25a32-mutation-in-a-patient-with-a-severe-neuromuscular-phenotype
#13
Debby M E I Hellebrekers, Suzanne C E H Sallevelt, Tom E J Theunissen, Alexandra T M Hendrickx, Ralph W Gottschalk, Janneke G J Hoeijmakers, Daphna D Habets, Jörgen Bierau, Kees G Schoonderwoerd, Hubert J M Smeets
In a 51-year-old patient of consanguineous parents with a severe neuromuscular phenotype of early-onset ataxia, myoclonia, dysarthria, muscle weakness and exercise intolerance, exome sequencing revealed a novel homozygous variant (c.-264_31delinsCTCACAAATGCTCA) in the mitochondrial FAD-transporter gene SLC25A32. Flavin adenine dinucleotide (FAD) is an essential co-factor for many mitochondrial enzymes and impaired mitochondrial FAD-transport was supported by a reduced oxidative phosphorylation complex II activity in the patient's muscle, decreased ATP production in fibroblasts, and a deficiency of mitochondrial FAD-dependent enzymes...
April 26, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28443506/catestain-a-master-regulator-of-cardiovascular-functions
#14
Sushil K Mahata, Malapaka Kiranmayi, Nitish R Mahapatra
Cardiovascular disease (CVD), the most common cause of death globally, accounts for ~30% of all deaths worldwide. Hypertension is a common contributor to morbidity and mortality from CVD. The plasma concentration of chromogranin A (CgA) is elevated in patients with CVD as well as patients with established human essential hypertension and heart failure (HF). In contrast, the plasma level of the CgA-derived peptide catestatin (CST) is diminished in human essential hypertension. Low conversion of CgA-to-CST has been associated with increased mortality in patients hospitalized with acute HF...
April 24, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28443245/overexpression-of-the-atpase-inhibitory-factor-1-favors-a-non-metastatic-phenotype-in-breast-cancer
#15
Lucía García-Ledo, Cristina Nuevo-Tapioles, Carmen Cuevas-Martín, Inmaculada Martínez-Reyes, Beatriz Soldevilla, Lucía González-Llorente, José M Cuezva
Partial suppression of mitochondrial oxidative phosphorylation and the concurrent activation of aerobic glycolysis is a hallmark of proliferating cancer cells. Overexpression of the ATPase inhibitory factor 1 (IF1), an in vivo inhibitor of the mitochondrial ATP synthase, is observed in most prevalent human carcinomas favoring metabolic rewiring to an enhanced glycolysis and cancer progression. Consistently, a high expression of IF1 in hepatocarcinomas and in carcinomas of the lung, bladder, and stomach and in gliomas is a biomarker of bad patient prognosis...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28443090/immunometabolic-regulations-mediated-by-coinhibitory-receptors-and-their-impact-on-t-cell-immune-responses
#16
REVIEW
Nikolaos Patsoukis, Jessica D Weaver, Laura Strauss, Christoph Herbel, Pankaj Seth, Vassiliki A Boussiotis
Host immunity provides wide spectrum protection that serves to eradicate pathogens and cancer cells, while maintaining self-tolerance and immunological homeostasis. Ligation of the T cell receptor (TCR) by antigen activates signaling pathways that coordinately induce aerobic glycolysis, mitochondrial activity, anabolic metabolism, and T effector cell differentiation. Activation of PI3K, Akt, and mTOR triggers the switch to anabolic metabolism by inducing transcription factors such as Myc and HIF1, and the glucose transporter Glut1, which is pivotal for the increase of glucose uptake after T cell activation...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28443065/hemoglobin-improved-protection-in-cultured-cerebral-cortical-astroglial-cells-inhibition-of-oxidative-stress-and-caspase-activation
#17
Fatma Amri, Ikram Ghouili, Marie-Christine Tonon, Mohamed Amri, Olfa Masmoudi-Kouki
Oxidative stress plays a major role in triggering astroglial cell death in diverse neuropathological conditions such as ischemia and neurodegenerative diseases. Numerous studies indicate that hemoglobin (Hb) is expressed in both resting and reactive glia cells, but nothing is known regarding a possible role of Hb on astroglial cell survival. Thus, the purpose of the present study was to investigate the potential glioprotective effect of Hb on hydrogen peroxide (H2O2)-induced oxidative stress and apoptosis in cultured rat astrocytes...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28443030/mitochondrial-reactive-oxygen-species-and-kidney-hypoxia-in-the-development-of-diabetic-nephropathy
#18
REVIEW
Tomas A Schiffer, Malou Friederich-Persson
The underlying mechanisms in the development of diabetic nephropathy are currently unclear and likely consist of a series of dynamic events from the early to late stages of the disease. Diabetic nephropathy is currently without curative treatments and it is acknowledged that even the earliest clinical manifestation of nephropathy is preceded by an established morphological renal injury that is in turn preceded by functional and metabolic alterations. An early manifestation of the diabetic kidney is the development of kidney hypoxia that has been acknowledged as a common pathway to nephropathy...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28442376/copper-sulfate-pretreatment-prevents-mitochondrial-electron-transport-chain-damage-and-apoptosis-against-mpp-induced-neurotoxicity
#19
Moisés Rubio-Osornio, Marisol Orozco-Ibarra, Araceli Díaz-Ruiz, Eduardo Brambila, Marie-Catherine Boll, Antonio Monroy-Noyola, Jorge Guevara, Sergio Montes, Camilo Ríos
Intrastriatal injection of 1-methyl-4-phenylpyridinium (MPP(+)) is considered a model to reproduce some biochemical alterations observed in Parkinson's disease (PD) patients. Among those alterations, inhibition of mitochondrial complex I activity, increased free radical production and reduced antioxidant responses have been reported. Copper (Cu) plays an important role in the metabolism and antioxidative responses through its participation as a cofactor in the cytochrome c oxidase enzyme (COX), Cu/Zn-superoxide dismutase (Cu/Zn-SOD), and metallothioneins...
April 22, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28442181/hospitalizations-for-mitochondrial-disease-across-the-lifespan-in-the-u-s
#20
Shana E McCormack, Rui Xiao, Todd J Kilbaugh, Michael Karlsson, Rebecca D Ganetzky, Zarazuela Zolkipli Cunningham, Amy Goldstein, Marni J Falk, Scott M Damrauer
IMPORTANCE: Mitochondrial disease is being diagnosed with increasing frequency. Although children with mitochondrial disease often have severe, life-limiting illnesses, many survive into adulthood. There is, however, limited information about the impact of mitochondrial disease on healthcare utilization in the U.S. across the lifespan. OBJECTIVES: To describe the characteristics of inpatient hospitalizations related to mitochondrial disease in the U.S., to identify patient-level clinical factors associated with in-hospital mortality, and to estimate the burden of hospitalizations on individual patients...
April 19, 2017: Molecular Genetics and Metabolism
keyword
keyword
54112
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"