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https://www.readbyqxmd.com/read/29767814/inborn-errors-of-coenzyme-a-metabolism-and-neurodegeneration
#1
Ivano Di Meo, Miryam Carecchio, Valeria Tiranti
Two inborn errors of coenzyme A (CoA) metabolism are responsible for distinct forms of neurodegeneration with brain iron accumulation (NBIA), a heterogeneous group of neurodegenerative diseases having as a common denominator iron accumulation mainly in the inner portion of globus pallidus. Pantothenate kinase-associated neurodegeneration (PKAN), an autosomal recessive disorder with progressive impairment of movement, vision and cognition, is the most common form of NBIA and is caused by mutations in the pantothenate kinase 2 gene (PANK2), coding for a mitochondrial enzyme, which phosphorylates vitamin B5 in the first reaction of the CoA biosynthetic pathway...
May 16, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29750731/movement-disorders-in-mitochondrial-disease-a-clinicopathological-correlation
#2
Irene H Flønes, Charalampos Tzoulis
PURPOSE OF REVIEW: The scope of this review is to give an updated account of movement disorders associated with mitochondrial disease, with a particular focus on recently discovered clinicopathological correlations. RECENT FINDINGS: Movement disorders are common clinical manifestations of mitochondrial diseases, in part because of the high vulnerability of neurons controlling motor circuits to mitochondrial respiratory dysfunction and energy failure. Intriguingly, the clinicopathological correlations of movement disorders in mitochondrial disease do not always conform to established neurophysiological knowledge...
May 9, 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29736331/patterns-of-genetic-structuring-at-the-northern-limits-of-the-australian-smelt-retropinna-semoni-cryptic-species-complex
#3
Md Rakeb-Ul Islam, Daniel J Schmidt, David A Crook, Jane M Hughes
Freshwater fishes often exhibit high genetic population structure due to the prevalence of dispersal barriers (e.g., waterfalls) whereas population structure in diadromous fishes tends to be weaker and driven by natal homing behaviour and/or isolation by distance. The Australian smelt (Retropinnidae: Retropinna semoni ) is a native fish with a broad distribution spanning inland and coastal drainages of south-eastern Australia. Previous studies have demonstrated variability in population genetic structure and movement behaviour (potamodromy, facultative diadromy, estuarine residence) across the southern part of its geographic range...
2018: PeerJ
https://www.readbyqxmd.com/read/29735120/movement-disorders-and-neurometabolic-diseases
#4
Celanie K Christensen, Laurence Walsh
Many inherited metabolic diseases or inborn errors of metabolism (IEM) cause movement disorders in children. This review focuses on chorea, dystonia, myoclonus, tremor, and parkinsonism. Broad neurometabolic categories commonly responsible for pediatric movement disorders include mitochondrial cytopathies, organic acidemias, mineral metabolism and transport disorders, neurotransmitter diseases, purine metabolism abnormalities, lipid storage conditions, and creatine metabolism dysfunction. Each movement disorder can be caused by many IEM and several of them can cause multiple movement abnormalities...
April 2018: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29727704/overexpression-of-nmnat-improves-the-adaption-of-health-span-in-aging-drosophila
#5
Xianchu Liu, Zhuo Xiang, Qiufang Li, Xiangcheng Ruan, Kaixin Xiong, Wen Dengtai, Lan Zheng
Nmnat is a stress response protein which has been involved in a variety of biological processes. However, the effects of Nmnat on aging have not yet been investigated. The present study revealed the effects of Nmnat on aging of Drosophila and uncovered its underlying mechanism. Therefore, the overexpression of Nmnat was established by arm/Gal4 system in Drosophila with an aim to determine the functions of Nmnat during aging process. In this study, our results showed Nmnat was a positive factor on lifespan and movement capacity, which was consistent on d-galactose induced aging acceleration...
May 1, 2018: Experimental Gerontology
https://www.readbyqxmd.com/read/29699918/cyclodextrins-or-cholesterol-loaded-cyclodextrins-a-better-choice-for-improved-cryosurvival-of-chicken-spermatozoa
#6
Agnieszka Partyka, Maciej Strojecki, Wojciech Niżański
This study was designed to test if treating chicken sperm with i) the cyclodextrins 2-hydroxypropyl-β-cyclodextrin (HBCD) and methyl-β-cyclodextrin (MBCD) alone improve fresh, liquid-stored and cryopreserved semen quality, or ii) cholesterol-loaded cyclodextrins (CLCs): 2-hydroxypropyl-β-cyclodextrin loaded with cholesterol (HCLC) and methyl-β-cyclodextrin loaded with cholesterol (MCLC) enhance chicken semen quality for application to assisted reproductive technologies. Three consecutive experiments were performed with different concentrations of additives: Exp...
April 21, 2018: Animal Reproduction Science
https://www.readbyqxmd.com/read/29686046/mcu-interacts-with-miro1-to-modulate-mitochondrial-functions-in-neurons
#7
Robert F Niescier, Kido Hong, Dongkeun Park, Kyung-Tai Min
Mitochondrial Ca2+ uptake is gated by the mitochondrial calcium uniplex, which is comprised of MCU, the Ca2+ pore-forming subunit of the complex, and its regulators. Ca2+ influx through MCU affects both mitochondrial function and movement in neurons, but its direct role in mitochondrial movement has not been explored. In this report, we show a link between MCU and Miro1, a membrane protein known to regulate mitochondrial movement. We find that MCU interacts with Miro1 through MCU's N-terminal domain, previously thought to be the mitochondrial targeting sequence...
April 23, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29653138/neuroprotective-action-of-4-hydroxyisophthalic-acid-against-paraquat-induced-motor-impairment-involves-amelioration-of-mitochondrial-damage-and-neurodegeneration-in-drosophila
#8
Niveditha S, T Shivanandappa
Neurodegenerative disorders including Parkinson's disease (PD) are believed to be caused by oxidative stress and mitochondrial dysfunction. Exposure to environmental agents such as pesticides has been implicated in the etiology of sporadic PD. Paraquat (PQ), a widely used herbicide, induces PD symptoms in laboratory animals including Drosophila. PQ acts as a free radical generator and induces oxidative damage, which is implicated in neuronal cell death. Drosophila model of PQ-induced PD offers a convenient tool for mechanistic studies and, to assess the neuroprotective potential of natural antioxidants...
April 10, 2018: Neurotoxicology
https://www.readbyqxmd.com/read/29651747/tauroursodeoxycholic-acid-improves-motor-symptoms-in-a-mouse-model-of-parkinson-s-disease
#9
Alexandra Isabel Rosa, Sara Duarte-Silva, Anabela Silva-Fernandes, Maria João Nunes, Andreia Neves Carvalho, Elsa Rodrigues, Maria João Gama, Cecília Maria Pereira Rodrigues, Patrícia Maciel, Margarida Castro-Caldas
Parkinson's disease (PD) is characterized by severe motor symptoms, and currently there is no treatment that retards disease progression or reverses damage prior to the time of clinical diagnosis. Tauroursodeoxycholic acid (TUDCA) is neuroprotective in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) mouse model of PD; however, its effect in PD motor symptoms has never been addressed. In the present work, an extensive behavior analysis was performed to better characterize the MPTP model of PD and to evaluate the effects of TUDCA in the prevention/improvement of mice phenotype...
April 12, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29644751/disease-modification-in-parkinson-s-disease-current-approaches-challenges-and-future-considerations
#10
REVIEW
Anthony E Lang, Alberto J Espay
The greatest unmet therapeutic need in Parkinson's disease is the development of treatment that slows the relentless progression of the neurodegenerative process. The concept of "disease modification" encompasses intervention types ranging from those designed to slow the underlying degeneration to treatments directed at regenerating or replacing lost neurons. To date all attempts to develop effective disease-modifying therapy have failed. Many reasons have been proposed for these failures including our rudimentary understanding of disease pathogenesis and the assumption that each targeted mechanisms of disease apply to most patients with the same clinical diagnosis...
April 11, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29644085/protein-molecular-modeling-shows-residue-t599-is-critical-to-wild-type-function-of-polg-and-description-of-a-novel-variant-associated-with-the-sando-phenotype
#11
John E Richter, Hector G Robles, Elizabeth Mauricio, Ahmed Mohammad, Paldeep S Atwal, Thomas R Caulfield
Sensory ataxic neuropathy with dysarthria and ophthalmoparesis (SANDO) is a rare phenotype resulting from pathogenic variants of mitochondrial DNA polymerase gamma ( POLG ). We modeled a novel POLG variant, T599P, that causes the SANDO phenotype and another variant at the same residue, p.T599E, to observe their effect on protein function and confirm the pathogenicity of T599P. Through neoteric molecular modeling techniques, we show that changes at the T599 residue position introduce extra rigidity into the surrounding helix-loop-helix, which places steric pressure on nearby nucleotides...
2018: Human Genome Variation
https://www.readbyqxmd.com/read/29628597/anesthetic-management-in-pediatric-patient-for-percutaneous-endoscopic-gastrostomy-with-mitochondrial-myopathy-leigh-syndrome
#12
Ebru Tarikçi Kiliç, Nelgin Gerenli, Mehmet Salim Akdemir, Necmi Onur Tastan, Egemen Atag
Leigh syndrome (LS) is a rare disease mainly affecting the central nervous system due to the abnormalities of mitochondrial energy generation and seen in early childhood with progressive loss of movement, mental abilities, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Anesthesia and surgery exacerbate the risks of aspiration, wheezing, and breathing difficulties. Tracheal irritability can be stimulated with the efforts of intubation. We report the anesthetic management of a rare case of an 11-year-old boy with a severe form of LS for percutaneous endoscopic gastrostomy insertion...
January 2018: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/29625080/the-effects-of-age-on-mitochondria-axonal-transport-and-axonal-degeneration-after-chronic-iop-elevation-using-a-murine-ocular-explant-model
#13
Elizabeth C Kimball, Joan L Jefferys, Mary E Pease, Ericka N Oglesby, Cathy Nguyen, Julie Schaub, Ian Pitha, Harry A Quigley
The purpose of this study was to compare younger and older mice after chronic intraocular pressure (IOP) elevation lasting up to 4 days with respect to mitochondrial density, structure, and movement, as well as axonal integrity, in an ex vivo explant model. We studied 2 transgenic mouse strains, both on a C57BL/6J background, one expressing yellow fluorescent protein (YFP) in selected axons and one expressing cyan fluorescent protein (CFP) in all mitochondria. Mice of 4 months or 14 months of age were exposed to chronic IOP by anterior chamber microbead injection for 14 h, 1, 3, or 4 days...
April 3, 2018: Experimental Eye Research
https://www.readbyqxmd.com/read/29624723/randomized-clinical-trial-of-rt001-early-signals-of-efficacy-in-friedreich-s-ataxia
#14
Theresa Zesiewicz, Frederic Heerinckx, Robert De Jager, Omid Omidvar, Marcus Kilpatrick, Jessica Shaw, Mikhail S Shchepinov
BACKGROUND: RT001 is a deuterated ethyl linoleate that inhibits lipid peroxidation and is hypothesized to reduce cellular damage and recover mitochondrial function in degenerative diseases such as Friedreich's ataxia. OBJECTIVE: To evaluate the safety, pharmacokinetics, and preliminary efficacy of RT001 in Friedreich's ataxia patients. DESIGN/METHODS: We conducted a phase I/II double-blind, comparator-controlled trial with 2 doses of RT001 in Friedreich's ataxia patients (9 subjects each cohort)...
April 6, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29623822/mass-spectrometric-analysis-of-proteins-of-l6-skeletal-muscle-cells-under-different-glucose-conditions-and-vitamin-d-supplementation
#15
Priyadarshini, Nancy Taneja
Protein expression during the exposure of the cells to different glucose concentrations may alter and can give vital information about the pathogenesis of T2D. Vitamin D deficiency is associated with various diseases and its supplementation can improve the diseased state. L6 skeletal muscle cells were exposed to different glucose (G) concentrations (0mM, 8mM, 16mM and 25mM) supplemented with vitamin D (VD) for 48 hours. Three distinct bands observed in SDS-PAGE in samples obtained from cells which were exposed to 8mM (G), 8mM (G) + VD and 16mM (G)...
April 6, 2018: Protein and Peptide Letters
https://www.readbyqxmd.com/read/29604224/mutations-in-vps13d-lead-to-a-new-recessive-ataxia-with-spasticity-and-mitochondrial-defects
#16
Eunju Seong, Ryan Insolera, Marija Dulovic, Erik-Jan Kamsteeg, Joanne Trinh, Norbert Brüggemann, Erin Sandford, Sheng Li, Ayse Bilge Ozel, Jun Z Li, Tamison Jewett, Anneke J A Kievit, Alexander Münchau, Vikram Shakkottai, Christine Klein, Catherine Collins, Katja Lohmann, Bart P van de Warrenburg, Margit Burmeister
OBJECTIVE: To identify novel causes of recessive ataxias, including spinocerebellar ataxia with saccadic intrusions, spastic ataxias and spastic paraplegia. METHODS: In an international collaboration, we independently performed exome sequencing in seven families with recessive ataxia and/or spastic paraplegia. To evaluate the role of VPS13D mutations, we evaluated a Drosophila knock-out model and investigated mitochondrial function in patient-derived fibroblast cultures...
March 31, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29602309/regenerative-abilities-of-mesenchymal-stem-cells-through-mitochondrial-transfer
#17
REVIEW
Swati Paliwal, Rituparna Chaudhuri, Anurag Agrawal, Sujata Mohanty
The past decade has witnessed an upsurge in studies demonstrating mitochondrial transfer as one of the emerging mechanisms through which mesenchymal stem cells (MSCs) can regenerate and repair damaged cells or tissues. It has been found to play a critical role in healing several diseases related to brain injury, cardiac myopathies, muscle sepsis, lung disorders and acute respiratory disorders. Several studies have shown that various mechanisms are involved in mitochondrial transfer that includes tunnel tube formation, micro vesicle formation, gap junctions, cell fusion and others modes of transfer...
March 30, 2018: Journal of Biomedical Science
https://www.readbyqxmd.com/read/29570846/neuroprotective-effects-of-creatine-in-the-cmvmjd135-mouse-model-of-spinocerebellar-ataxia-type-3
#18
Sara Duarte-Silva, Andreia Neves-Carvalho, Carina Soares-Cunha, Joana M Silva, Andreia Teixeira-Castro, Rita Vieira, Anabela Silva-Fernandes, Patrícia Maciel
BACKGROUND AND OBJECTIVE: Mitochondrial dysfunction has been implicated in several neurodegenerative diseases. Creatine administration increases concentration of the energy buffer phosphocreatine, exerting protective effects in the brain. We evaluate whether a creatine-enriched diet would be beneficial for a mouse model of spinocerebellar ataxia type 3, a genetically defined neurodegenerative disease for which no treatment is available. METHODS: We performed 2 independent preclinical trials using the CMVMJD135 mouse model (treating 2 groups of animals with different disease severity) and wild-type mice, to which 2% creatine was provided for 19 (preclinical trial 1) or 29 (preclinical trial 2) weeks, starting at a presymptomatic age...
March 23, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29566165/leydig-cells-formation-function-and-regulation
#19
Barry R Zirkin, Vassilios Papadopoulos
Herein we summarize important discoveries made over many years about Leydig cell function and regulation. During the fetal period, fetal Leydig cells produce the high levels of androgen (testosterone or androstenedione, depending upon the species) required for differentiation of male genitalia and brain masculinization. Androgen production declines with loss of these cells, reaching a nadir at postpartum. Testosterone then gradually increases to high levels with adult Leydig cell development from stem cells...
March 16, 2018: Biology of Reproduction
https://www.readbyqxmd.com/read/29551757/impacts-of-late-quaternary-environmental-change-on-the-long-tailed-ground-squirrel-urocitellus-undulatus-in-mongolia
#20
Bryan S McLean, Batsaikhan Nyamsuren, Andrey Tchabovsky, Joseph A Cook
Impacts of Quaternary environmental changes on mammal faunas of central Asia remain poorly understood due to a lack of geographically comprehensive phylogeographic sampling for most species. To help address this knowledge gap, we conducted the most extensive molecular analysis to date of the long-tailed ground squirrel (Urocitellus undulatus Pallas 1778) in Mongolia, a country that comprises the southern core of this species' range. Drawing on material from recent collaborative field expeditions, we genotyped 128 individuals at 2 mitochondrial genes (cytochrome b and cytochrome oxidase I; 1 797 bp total)...
March 8, 2018: Zoological Research
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