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https://www.readbyqxmd.com/read/28527629/p62-sequestosome-1-knockout-delays-neurodegeneration-induced-by-drp1-loss
#1
Tatsuya Yamada, Yoshihiro Adachi, Toru Yanagawa, Miho Iijima, Hiromi Sesaki
Purkinje neurons, one of the largest neurons in the brain, are critical for controlling body movements, and the dysfunction and degeneration of these cells cause ataxia. Purkinje neurons require a very efficient energy supply from mitochondria because of their large size and extensive dendritic arbors. We have previously shown that mitochondrial division mediated by dynamin-related protein 1 (Drp1) is critical for the development and survival of Purkinje neurons. Drp1 deficiency has been associated with one of the major types of ataxia: autosomal recessive spastic ataxia of Charlevoix Saguenay...
May 17, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28507584/effects-of-adenosine-receptor-antagonists-in-mptp-mouse-model-of-parkinson-s-disease-mitochondrial-dna-integrity
#2
Soha S Essawy, Mona Kamal Tawfik, Horya Erfan Korayem
INTRODUCTION: In Parkinson's disease (PD), compelling data indicate a functional link between adenosine/dopamine receptors and the progression of the neurodegenerative process. The present study was carried out to evaluate the effect of the non-selective adenosine receptor (ADR) antagonist caffeine, as well as the selective antagonists 8-cyclopentyl-1,3-dipropylxanthine (DPCPX), an ADRsA1 antagonist, and ((E)-1,3-diethyl-8-(3,4-dimethoxystyryl)-7-methyl-3,7-dihydro-1H-purine-2,6-dione) (KW-6002), an ADRsA2A antagonist, on the prevention of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced Parkinsonism in mice...
April 1, 2017: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/28506426/heterogeneity-of-fhf1-related-phenotype-novel-case-with-early-onset-severe-attacks-of-apnea-partial-mitochondrial-respiratory-chain-complex-ii-deficiency-neonatal-onset-seizures-without-neurodegeneration
#3
Nathalie Villeneuve, Affef Abidi, Pierre Cacciagli, Cécile Mignon-Ravix, Brigitte Chabrol, Laurent Villard, Mathieu Milh
INTRODUCTION/OBJECTIVES: We report the case of a child prospectively followed in our institution for a severe, neonatal onset epilepsy presenting with severe attacks of apnea that were not initially recognized as seizure since they were not associated with any abnormal movement and since interictal EEG was normal. Recording of attacks using prolonged video-EEG recording allowed to confirm the diagnosis of epileptic seizures. RESULTS: Using whole exome sequencing we found a de novo heterozygous, missense mutation of FHF1 (p...
April 29, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28498971/deletion-of-rhoa-in-progesterone-receptor-expressing-cells-leads-to-luteal-insufficiency-and-infertility-in-female-mice
#4
Ahmed E El Zowalaty, Rong Li, Yi Zheng, John P Lydon, Francesco J DeMayo, Xiaoqin Ye
RhoA is widely expressed throughout the female reproductive system. To assess its role in progesterone receptor (Pgr) expressing cells, we generated RhoA conditional knockout mice RhoAd/d (RhoAf/f-Pgr-Cre+/-). RhoAd/d females had comparable mating activity, serum LH, prolactin, and estradiol levels and ovulation with control but were infertile with progesterone insufficiency, indicating impaired steroidogenesis in RhoAd/d corpus luteum (CL). RhoA was highly expressed in wild type luteal cells and conditionally deleted in RhoAd/d CL...
May 11, 2017: Endocrinology
https://www.readbyqxmd.com/read/28495532/new-insights-into-the-role-of-mitochondrial-calcium-homeostasis-in-cell-migration
#5
Vincent Paupe, Julien Prudent
Mitochondria are dynamic organelles involved in numerous physiological functions. Beyond their function in ATP production, mitochondria regulate cell death, reactive oxygen species (ROS) generation, immunity and metabolism. Mitochondria also play a key role in the buffering of cytosolic calcium, and calcium transported into the matrix regulates mitochondrial metabolism. Recently, the identification of the mitochondrial calcium uniporter (MCU) and associated regulators has allowed the characterization of new physiological roles for calcium in both mitochondrial and cellular homeostasis...
May 8, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28484149/the-genetic-diversity-of-d-loop-sequences-in-eastern-bent-winged-bats-miniopterus-fuliginosus-living-in-wakayama-prefecture-japan
#6
Keisuke Iida, Ryosuke Kobayashi, Yupadee Hengjan, Nao Nagata, Kenzo Yonemitsu, Mitsuo Nunome, Ryusei Kuwata, Kazuo Suzuki, Kenji Ichiyanagi, Ken Maeda, Yasushige Ohmori, Eiichi Hondo
The eastern bent-winged bat (Miniopterus fuliginosus) is an insectivorous bat that lives in the caves, throughout Japan. The bats aggregate in cave in populations of tens to thousands of individuals. We examined the mitochondrial D-loop sequences of bats in Wakayama, Japan, and divided them into 35 haplotypes. The sequences of 3 haplotypes in Wakayama were the same as those of 10 Miniopterus fuliginosus individuals living in China. Given the substitution rate of the D-loop region, we speculated that the bats had moved between Japan and China within the last 16,000 years...
May 8, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28479995/genetic-structure-among-greater-white-fronted-goose-populations-of-the-pacific-flyway
#7
Craig R Ely, Robert E Wilson, Sandra L Talbot
An understanding of the genetic structure of populations in the wild is essential for long-term conservation and stewardship in the face of environmental change. Knowledge of the present-day distribution of genetic lineages (phylogeography) of a species is especially important for organisms that are exploited or utilize habitats that may be jeopardized by human intervention, including climate change. Here, we describe mitochondrial (mtDNA) and nuclear genetic (microsatellite) diversity among three populations of a migratory bird, the greater white-fronted goose (Anser albifrons), which breeds discontinuously in western and southwestern Alaska and winters in the Pacific Flyway of North America...
May 2017: Ecology and Evolution
https://www.readbyqxmd.com/read/28476494/diversity-and-phylogeography-of-northeast-asian-brown-frogs-allied-to-rana-dybowskii-anura-ranidae
#8
Bao-Tian Yang, Yu Zhou, Mi-Sook Min, Masafumi Matsui, Bing-Jun Dong, Pi-Peng Li, Jonathan J Fong
We investigated the species diversity and phylogeography of the Northeast Asian brown frogs allied to Rana dybowskii (the R. dybowskii species complex: R. dybowskii, R. pirica, and R. uenoi) using four mitochondrial and three nuclear loci. Phylogenetic analyses confirmed the existence of three distinct species in this complex; using extensive molecular data, we confirm the validity of Rana uenoi recognized as a distinct species, and infer R. dybowskii and R. pirica to be sister species. Also, we included populations from previously unsampled regions in Northeast China, and identified them to be R...
May 2, 2017: Molecular Phylogenetics and Evolution
https://www.readbyqxmd.com/read/28473328/tnf%C3%AE-decreases-mitochondrial-movement-in-human-airway-smooth-muscle
#9
Philippe F Delmotte, Vanessa A Zavaletta, Michael A Thompson, Y S Prakash, Gary C Sieck
In airway smooth muscle (ASM) cells, excitation-contraction coupling is accomplished via a cascade of events which connect an elevation of cytosolic Ca(2+) concentration ([Ca(2+)]cyt) with cross-bridge attachment and ATP-consuming mechanical work. Excitation-energy coupling is mediated by linking the elevation in [Ca(2+)]cyt to an increase in mitochondrial Ca(2+) ([Ca(2+)]mito) which in turn stimulates ATP production. Mitochondria proximity with the sarcoplasmic reticulum (SR) and plasma membrane is thought to be an important mechanism to facilitate mitochondrial Ca(2+) uptake...
May 4, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28461171/connecting-mitochondrial-dynamics-and-life-or-death-events-via-bcl-2-family-proteins
#10
REVIEW
Abdel Aouacheria, Stephen Baghdiguian, Heather M Lamb, Jason D Huska, Fernando J Pineda, J Marie Hardwick
The morphology of a population of mitochondria is the result of several interacting dynamical phenomena, including fission, fusion, movement, elimination and biogenesis. Each of these phenomena is controlled by underlying molecular machinery, and when defective can cause disease. New understanding of the relationships between form and function of mitochondria in health and disease is beginning to be unraveled on several fronts. Studies in mammals and model organisms have revealed that mitochondrial morphology, dynamics and function appear to be subject to regulation by the same proteins that regulate apoptotic cell death...
April 28, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28455141/identification-of-potential-therapeutic-compounds-for-parkinson-s-disease-using-drosophila-and-human-cell-models
#11
Francisco José Sanz, Cristina Solana-Manrique, Verónica Muñoz-Soriano, Pablo Calap-Quintana, María Dolores Moltó, Nuria Paricio
Parkinson's disease (PD) is the second most common neurodegenerative disorder after Alzheimer's disease. It is caused by a loss of dopaminergic neurons in the substantia nigra pars compacta, leading to a decrease in dopamine levels in the striatum and thus producing movement impairment. Major physiological causes of neurodegeneration in PD are oxidative stress (OS) and mitochondrial dysfunction; these pathophysiological changes can be caused by both genetic and environmental factors. Although most PD cases are sporadic, it has been shown that 5-10% of them are familial forms caused by mutations in certain genes...
April 25, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28453513/genetic-diversity-and-population-structure-of-the-tsetse-fly-glossina-fuscipes-fuscipes-diptera-glossinidae-in-northern-uganda-implications-for-vector-control
#12
Robert Opiro, Norah P Saarman, Richard Echodu, Elizabeth A Opiyo, Kirstin Dion, Alexis Halyard, Augustine W Dunn, Serap Aksoy, Adalgisa Caccone
Uganda is the only country where the chronic and acute forms of human African Trypanosomiasis (HAT) or sleeping sickness both occur and are separated by < 100 km in areas north of Lake Kyoga. In Uganda, Glossina fuscipes fuscipes is the main vector of the Trypanosoma parasites responsible for these diseases as well for the animal African Trypanosomiasis (AAT), or Nagana. We used highly polymorphic microsatellite loci and a mitochondrial DNA (mtDNA) marker to provide fine scale spatial resolution of genetic structure of G...
April 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28450076/local-energy-on-demand-are-spontaneous-astrocytic-ca-2-microdomains-the-regulatory-unit-for-astrocyte-neuron-metabolic-cooperation
#13
REVIEW
Martin Oheim, Elke Schmidt, Johannes Hirrlinger
Astrocytes are a neural cell type critically involved in maintaining brain energy homeostasis as well as signaling. Like neurons, astrocytes are a heterogeneous cell population. Cortical astrocytes show a complex morphology with a highly branched aborization and numerous fine processes ensheathing the synapses of neighboring neurons, and typically extend one process connecting to blood vessels. Recent studies employing genetically encoded fluorescent calcium (Ca(2+)) indicators have described 'spontaneous' localized Ca(2+)-transients in the astrocyte periphery that occur asynchronously, independently of signals in other parts of the cells, and that do not involve somatic Ca(2+) transients; however, neither it is known whether these Ca(2+)-microdomains occur at or near neuronal synapses nor have their molecular basis nor downstream effector(s) been identified...
April 24, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28445716/trumping-neurodegeneration-targeting-common-pathways-regulated-by-autosomal-recessive-parkinson-s-disease-genes
#14
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/28440746/dual-interaction-of-scaffold-protein-tim44-of-mitochondrial-import-motor-with-channel-forming-translocase-subunit-tim23
#15
See-Yeun Ting, Nicholas L Yan, Brenda A Schilke, Elizabeth A Craig
Proteins destined for the mitochondrial matrix are targeted to the inner membrane Tim17/23 translocon by their presequences. Inward movement is driven by the matrix-localized, Hsp70-based motor. The scaffold Tim44, interacting with the matrix face of the translocon, recruits other motor subunits and binds incoming presequence. The basis of these interactions and their functional relationships remains unclear. Using site-specific in vivo crosslinking and genetic approaches in Saccharomyces cerevisiae, we found that both domains of Tim44 interact with the major matrix-exposed loop of Tim23, with the C-terminal domain (CTD) binding Tim17 as well...
April 25, 2017: ELife
https://www.readbyqxmd.com/read/28433109/pathogenesis-of-wilson-disease
#16
Ivo Florin Scheiber, Radan Brůha, Petr Dušek
Wilson disease is an autosomal-recessive disorder originating from a genetic defect in the copper-transporting ATPase ATP7B that is required for biliary copper secretion and loading of ceruloplasmin with copper. Impaired ATP7B function in Wilson disease results in excessive accumulation of copper in liver, brain, and other tissues. Toxic copper deposits may induce oxidative stress, modify expression of genes, directly inhibit proteins, and impair mitochondrial function, leading to hepatic, neuropsychiatric, renal, musculoskeletal, and other symptoms...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28424608/blood-transcriptomic-meta-analysis-identifies-dysregulation-of-hemoglobin-and-iron-metabolism-in-parkinson-disease
#17
Jose A Santiago, Judith A Potashkin
Disrupted iron metabolism has been implicated in the pathogenesis of Parkinson's disease (PD), a progressive neurodegenerative disorder that severely affects movement and coordination, yet the molecular mechanisms underlying this association remain unknown. To this end, we performed a transcriptomic meta-analysis of four blood microarrays in PD. We observed a significant downregulation of genes related to hemoglobin including, hemoglobin delta (HBD), alpha hemoglobin stabilizing protein (ASHP), genes implicated in iron metabolism including, solute carrier family 11 member 2 (SLC11A2), ferrochelatase (FECH), and erythrocyte-specific genes including erythrocyte membrane protein (EPB42), and 5'-aminolevulinate synthase 2 (ALAS2)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28414942/coenzyme-q10-protects-against-statin-induced-myotoxicity-in-zebrafish-larvae-danio-rerio
#18
Rand Pasha, Thomas W Moon
3-Hydroxy-3-methylglutaryl-CoA reductase (HMGCR) is the rate-limiting enzyme of the mevalonic acid pathway and is required for cholesterol biosynthesis and the synthesis of Coenzyme Q10 (CoQ10). Statins inhibit HMGCR, thus inhibiting the downstream products of this pathway including the biosynthesis of decaprenyl-pyrophosphate that is critical for the synthesis of Coenzyme Q10 (CoQ10). We show that zebrafish (Danio rerio) larvae treated in tank water with Atorvastatin (ATV; Lipitor) exhibited movement alterations and reduced whole body tissue metabolism...
April 1, 2017: Environmental Toxicology and Pharmacology
https://www.readbyqxmd.com/read/28414059/a-mouse-ocular-explant-model-that-enables-the-study-of-living-optic-nerve-head-events-after-acute-and-chronic-intraocular-pressure-elevation-focusing-on-retinal-ganglion-cell-axons-and-mitochondria
#19
Elizabeth C Kimball, Mary E Pease, Matthew R Steinhart, Ericka N Oglesby, Ian Pitha, Cathy Nguyen, Harry A Quigley
We developed an explant model of the mouse eye and optic nerve that facilitates the study of retinal ganglion cell axons and mitochondria in the living optic nerve head (ONH) in an ex vivo environment. Two transgenic mouse strains were used, one expressing yellow fluorescent protein in selected axons and a second strain expressing cyan fluorescent protein in all mitochondria. We viewed an explanted mouse eye and optic nerve by laser scanning microscopy at and behind the ONH, the site of glaucoma injury. Explants from previously untreated mice were studied with the intraocular pressure (IOP) set artificially at normal or elevated levels for several hours...
April 14, 2017: Experimental Eye Research
https://www.readbyqxmd.com/read/28410388/tiger-on-the-prowl-invasion-history-and-spatio-temporal-genetic-structure-of-the-asian-tiger-mosquito-aedes-albopictus-skuse-1894-in-the-indo-pacific
#20
Andrew J Maynard, Luke Ambrose, Robert D Cooper, Weng K Chow, Joseph B Davis, Mutizwa O Muzari, Andrew F van den Hurk, Sonja Hall-Mendelin, Jeomhee M Hasty, Thomas R Burkot, Michael J Bangs, Lisa J Reimer, Charles Butafa, Neil F Lobo, Din Syafruddin, Yan Naung Maung Maung, Rohani Ahmad, Nigel W Beebe
BACKGROUND: Within the last century, increases in human movement and globalization of trade have facilitated the establishment of several highly invasive mosquito species in new geographic locations with concurrent major environmental, economic and health consequences. The Asian tiger mosquito, Aedes albopictus, is an extremely invasive and aggressive daytime-biting mosquito that is a major public health threat throughout its expanding range. METHODOLOGY/PRINCIPAL FINDINGS: We used 13 nuclear microsatellite loci (on 911 individuals) and mitochondrial COI sequences to gain a better understanding of the historical and contemporary movements of Ae...
April 2017: PLoS Neglected Tropical Diseases
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