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Mitochondrial movement

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https://www.readbyqxmd.com/read/27924223/mitochondrial-permeability-transition-pore-induction-is-linked-to-formation-of-the-complex-of-atpase-c-subunit-polyhydroxybutyrate-and-inorganic-polyphosphate
#1
P A Elustondo, M Nichols, A Negoda, A Thirumaran, E Zakharian, G S Robertson, E V Pavlov
Mitochondrial permeability transition pore (mPTP) opening allows free movement of ions and small molecules leading to mitochondrial membrane depolarization and ATP depletion that triggers cell death. A multi-protein complex of the mitochondrial ATP synthase has an essential role in mPTP. However, the molecular identity of the central 'pore' part of mPTP complex is not known. A highly purified fraction of mammalian mitochondria containing C-subunit of ATPase (C-subunit), calcium, inorganic polyphosphate (polyP) and polyhydroxybutyrate (PHB) forms ion channels with properties that resemble the native mPTP...
2016: Cell Death Discovery
https://www.readbyqxmd.com/read/27903293/personalized-medicine-approach-confirms-a-milder-case-of-abat-deficiency
#2
A Besse, A K Petersen, J V Hunter, V Appadurai, S R Lalani, P E Bonnen
ABAT deficiency (OMIM 613163) is a rare inborn error of metabolism caused by recessive variants in the gene 4-aminobutyric acid transaminase (ABAT), which is responsible for both the catalysis of GABA and maintenance of nucleoside pools in the mitochondria. To date, only a few patients have been reported worldwide. Their clinical presentation has been remarkably consistent with primary features of severe psychomotor retardation, encephalopathy, hypotonia, and infantile-onset refractory epilepsy. We report a new case of ABAT deficiency that marks an important departure from previous clinical findings...
December 1, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27832395/mitoluhmes-an-engineered-neuronal-cell-line-for-the-analysis-of-the-motility-of-mitochondria
#3
Tomasz M Stępkowski, Sylwia Męczyńska-Wielgosz, Marcin Kruszewski
Perturbations in the transport of mitochondria and their quality control in neuronal cells underlie many types of neurological pathologies, whereas systems enabling convenient analysis of mitochondria behavior in cellular models of neurodegenerative diseases are limited. In this study, we present a modified version of lund human mesencephalic cells, mitoLUHMES, expressing GFP and mitochondrially targeted DsRed2 fluorescent proteins, intended for in vitro analysis of mitochondria trafficking by real-time fluorescence microscopy...
November 10, 2016: Cellular and Molecular Neurobiology
https://www.readbyqxmd.com/read/27826120/mitochondrial-oxidative-phosphorylation-disorders-in-children-phenotypic-genotypic-and-biochemical-correlations-in-85-patients-from-south-india
#4
Kothari Sonam, Parayil Sankaran Bindu, M M Srinivas Bharath, Periyasamy Govindaraj, Narayanappa Gayathri, Hanumanthapura R Arvinda, Shwetha Chiplunkar, Madhu Nagappa, Sanjib Sinha, Nahid Akhtar Khan, Vandana Nunia, Arumugam Paramasivam, Kumarasamy Thangaraj, Arun B Taly
Mitochondrial oxidative phosphorylation (OXPHOS) disorders accounts for a variety of neuromuscular disorders in children. In this study mitochondrial respiratory chain enzymes were assayed in muscle tissue in a large cohort of children with varied neuromuscular presentations from June 2011 to December 2013. The biochemical enzyme deficiencies were correlated with the phenotypes, magnetic resonance imaging, histopathology and genetic findings to reach a final diagnosis. There were 85 children (mean age: 6.9±4...
November 5, 2016: Mitochondrion
https://www.readbyqxmd.com/read/27812405/spatial-and-temporal-genetic-homogeneity-of-the-monterey-spanish-mackerel-scomberomorus-concolor-in-the-gulf-of-california
#5
Erika Magallón-Gayón, Pindaro Diaz-Jaimes, Manuel Uribe-Alcocer
The genetic homogeneity of the Monterey Spanish mackerel Scomberomorus concolor population in the Gulf of California was confirmed using nine nuclear microsatellite loci in combination with mitochondrial cytochrome b gene sequences. Samples were collected from the upper and central Gulf areas, representing the two main biogeographical regions of the Gulf. The analyses support the existence of a single panmictic population of S. concolor inhabiting the Gulf of California which in terms of fishery management represents a single genetic stock...
2016: PeerJ
https://www.readbyqxmd.com/read/27810319/gene-flow-for-echinococcus-granulosus-metapopulations-determined-by-mitochondrial-sequences-a-reliable-approach-for-reflecting-epidemiological-drift-of-parasite-among-neighboring-countries
#6
Mahmoud Mahami-Oskouei, Azam Kaseb-Yazdanparast, Adel Spotin, Abbas Shahbazi, Mohammad Adibpour, Ehsan Ahmadpour, Nader Ghabouli-Mehrabani
In genetic diversity and population structure of Echinococcus granulosus, the gene flow can illustrate how the Echinococcus isolates have epidemiologically drifted among endemic neighboring countries. 51 isolates of hydatid cysts were collected from human, dog, cattle and sheep in northwest Iran, where placed co-border with Turkey. DNA samples were extracted, amplified and subjected to sequence analysis of NADH dehydrogenase subunit 1 (nad1) and cytochrome oxidase subunit 1 (cox1) genes. As well, sequences of Echinococcus at east to the southeast regions of Turkey were retrieved from GenBank database for the cox1 gene...
October 31, 2016: Experimental Parasitology
https://www.readbyqxmd.com/read/27809277/the-neuroprotective-properties-of-hericium-erinaceus-in-glutamate-damaged-differentiated-pc12-cells-and-an-alzheimer-s-disease-mouse-model
#7
Junrong Zhang, Shengshu An, Wenji Hu, Meiyu Teng, Xue Wang, Yidi Qu, Yang Liu, Ye Yuan, Di Wang
Hericium erinaceus, an edible and medicinal mushroom, displays various pharmacological activities in the prevention of dementia in conditions such as Parkinson's and Alzheimer's disease. The present study explored the neuroprotective effects of H. erinaceus mycelium polysaccharide-enriched aqueous extract (HE) on an l-glutamic acid (l-Glu)-induced differentiated PC12 (DPC12) cellular apoptosis model and an AlCl₃ combined with d-galactose-induced Alzheimer's disease mouse model. The data revealed that HE successfully induced PC12 cell differentiation...
November 1, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27807421/cancer-and-chemotherapy-contribute-to-muscle-loss-by-activating-common-signaling-pathways
#8
Rafael Barreto, Giorgia Mandili, Frank A Witzmann, Francesco Novelli, Teresa A Zimmers, Andrea Bonetto
Cachexia represents one of the primary complications of colorectal cancer due to its effects on depletion of muscle and fat. Evidence suggests that chemotherapeutic regimens, such as Folfiri, contribute to cachexia-related symptoms. The purpose of the present study was to investigate the cachexia signature in different conditions associated with severe muscle wasting, namely Colon-26 (C26) and Folfiri-associated cachexia. Using a quantitative LC-MS/MS approach, we identified significant changes in 386 proteins in the quadriceps muscle of Folfiri-treated mice, and 269 proteins differentially expressed in the C26 hosts (p < 0...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27791192/structure-of-the-mitochondrial-atp-synthase-from-pichia-angusta-determined-by-electron-cryo-microscopy
#9
Kutti R Vinothkumar, Martin G Montgomery, Sidong Liu, John E Walker
The structure of the intact monomeric ATP synthase from the fungus, Pichia angusta, has been solved by electron cryo-microscopy. The structure provides insights into the mechanical coupling of the transmembrane proton motive force across mitochondrial membranes in the synthesis of ATP. This mechanism requires a strong and integral stator, consisting of the catalytic α3β3-domain, peripheral stalk, and, in the membrane domain, subunit a and associated supernumerary subunits, kept in contact with the rotor turning at speeds up to 350 Hz...
October 24, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27775167/genetic-diversity-and-phylogeographic-structure-of-bactrian-camels-shown-by-mitochondrial-sequence-variations
#10
L Ming, L Yi, R Sa, Z X Wang, Z Wang, R Ji
The Bactrian camel includes various domestic (Camelus bactrianus) and wild (Camelus ferus) breeds that are important for transportation and for their nutritional value. However, there is a lack of extensive information on their genetic diversity and phylogeographic structure. Here, we studied these parameters by examining an 809-bp mtDNA fragment from 113 individuals, representing 11 domestic breeds, one wild breed and two hybrid individuals. We found 15 different haplotypes, and the phylogenetic analysis suggests that domestic and wild Bactrian camels have two distinct lineages...
October 24, 2016: Animal Genetics
https://www.readbyqxmd.com/read/27774660/the-influence-of-benign-prostatic-hyperplasia-on-sperm-morphological-features-and-sperm-dna-integrity-in-dogs
#11
R B Flores, Dsr Angrimani, B R Rui, M M Brito, R A Abreu, C I Vannucchi
Benign prostatic hyperplasia (BPH) has a high incidence in older intact dogs. Due to the increased prostatic oxidative stress and hormonal imbalance of BPH, sperm damage can arise, such as sperm morphological alterations and DNA fragmentation. This study aimed to compare the reproductive potential of healthy dogs and those affected by benign prostatic hyperplasia. Ten dogs were assigned to two experimental groups: dogs without BPH (control; n = 5) and dogs diagnosed with BPH (n = 5), based on clinical signs and ultrasonographic findings...
October 24, 2016: Reproduction in Domestic Animals, Zuchthygiene
https://www.readbyqxmd.com/read/27746072/comparative-phylogeography-of-african-fruit-bats-chiroptera-pteropodidae-provide-new-insights-into-the-outbreak-of-ebola-virus-disease-in-west-africa-2014-2016
#12
Alexandre Hassanin, Nicolas Nesi, Julie Marin, Blaise Kadjo, Xavier Pourrut, Éric Leroy, Guy-Crispin Gembu, Prescott Musaba Akawa, Carine Ngoagouni, Emmanuel Nakouné, Manuel Ruedi, Didier Tshikung, Célestin Pongombo Shongo, Céline Bonillo
Both Ebolavirus and Marburgvirus were detected in several fruit bat species of the family Pteropodidae, suggesting that this taxon plays a key role in the life cycle of filoviruses. After four decades of Zaire Ebolavirus (ZEBOV) outbreaks in Central Africa, the virus was detected for the first time in West Africa in 2014. To better understand the role of fruit bats as potential reservoirs and circulating hosts between Central and West Africa, we examine here the phylogeny and comparative phylogeography of Pteropodidae...
November 2016: Comptes Rendus Biologies
https://www.readbyqxmd.com/read/27732084/rabl2-is-required-for-hepatic-fatty-acid-homeostasis-and-its-dysfunction-leads-to-steatosis-and-a-diabetes-like-state
#13
Jennifer Chi Yi Lo, Anne E O'Connor, Zane B Andrews, Camden Lo, Tony Tiganis, Matthew J Watt, Moira K O'Bryan
Fatty liver, or hepatic steatosis, is an alarmingly common pathology in western societies, in large part because if left unheeded, it can lead to life-threatening forms of nonalcoholic fatty liver disease, including nonalcoholic steatohepatitis, fibrosis, cirrhosis, and hepatocellular carcinoma. As such, it is essential that we attain a greater understanding of the pathways that control energy partitioning in the liver and ultimately how they are impacted by environmental factors. Here, we define the essential requirement for a member of the Ras-related protein in the brain (RAB)-like (RABL) clade of small GTPases, RABL2, in fatty acid metabolism including in microtubule-associated mitochondrial movement within the liver...
December 2016: Endocrinology
https://www.readbyqxmd.com/read/27723783/increase-in-cardiac-ischemia-reperfusion-injuries-in-opa1-mouse-model
#14
Sophie Le Page, Marjorie Niro, Jérémy Fauconnier, Laura Cellier, Sophie Tamareille, Abdallah Gharib, Arnaud Chevrollier, Laurent Loufrani, Céline Grenier, Rima Kamel, Emmanuelle Sarzi, Alain Lacampagne, Michel Ovize, Daniel Henrion, Pascal Reynier, Guy Lenaers, Delphine Mirebeau-Prunier, Fabrice Prunier
BACKGROUND: Recent data suggests the involvement of mitochondrial dynamics in cardiac ischemia/reperfusion (I/R) injuries. Whilst excessive mitochondrial fission has been described as detrimental, the role of fusion proteins in this context remains uncertain. OBJECTIVES: To investigate whether Opa1 (protein involved in mitochondrial inner-membrane fusion) deficiency affects I/R injuries. METHODS AND RESULTS: We examined mice exhibiting Opa1delTTAG mutations (Opa1+/-), showing 70% Opa1 protein expression in the myocardium as compared to their wild-type (WT) littermates...
2016: PloS One
https://www.readbyqxmd.com/read/27709644/a-case-of-hypopituitarism-accompanying-kearns-sayre-syndrome-treated-with-human-chorionic-gonadotropin-a-case-report-and-literature-review
#15
Y-X Kang, Y-J Wang, Q Zhang, X-H Pang, W Gu
Kearns-Sayre syndrome (KSS) is a disorder caused by mutations in mitochondrial DNA. Here, we report an unusual case of Kearns-Sayre syndrome accompanied by hypopituitarism (deficiencies in reproductive and growth hormones). A 20-year-old male presented with growth retardation for the last 8 years, as well as the following findings: short stature, delayed puberty, myasthenia, an extraocular movement deficit, drooping eyelids, pectus carinatum and scoliosis. Cerebral enhanced magnetic resonance imaging revealed dysplasias of the pituitary, white matter and cerebellum...
October 6, 2016: Andrologia
https://www.readbyqxmd.com/read/27696420/plasticity-in-mitochondrial-cristae-density-allows-metabolic-capacity-modulation-in-human-skeletal-muscle
#16
Joachim Nielsen, Kasper D Gejl, Martin Hey-Mogensen, Hans-Christer Holmberg, Charlotte Suetta, Peter Krustrup, Coen P H Elemans, Niels Ørtenblad
KEY POINTS: In human skeletal muscles, the current view is that the capacity for mitochondrial energy production, and thus endurance capacity, is set by the mitochondria volume. However, increasing the mitochondrial inner membrane surface comprises an alternative mechanism for increasing the energy production capacity. In the present study, we show that mitochondrial inner membranes in leg muscles of endurance-trained athletes have an increased ratio of surface per mitochondrial volume...
October 3, 2016: Journal of Physiology
https://www.readbyqxmd.com/read/27688826/altered-striatocerebellar-metabolism-and-systemic-inflammation-in-parkinson-s-disease
#17
Chiun-Chieh Yu, Meng-Hsiang Chen, Cheng-Hsien Lu, Yung-Cheng Huang, Hsiu-Ling Chen, Nai-Wen Tsai, Hung-Chen Wang, I-Hsiao Yang, Shau-Hsuan Li, Wei-Che Lin
Parkinson's disease (PD) is the most second common neurodegenerative movement disorder. Neuroinflammation due to systemic inflammation and elevated oxidative stress is considered a major factor promoting the pathogenesis of PD, but the relationship of structural brain imaging parameters to clinical inflammatory markers has not been well studied. Our aim was to evaluate the association of magnetic resonance spectroscopy (MRS) measures with inflammatory markers. Blood samples were collected from 33 patients with newly diagnosed PD and 30 healthy volunteers...
2016: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/27683074/sdha-mutation-with-dominant-transmission-results-in-complex-ii-deficiency-with-ocular-cardiac-and-neurologic-involvement
#18
Carolina Courage, Christopher B Jackson, Dagmar Hahn, Liliya Euro, Jean-Marc Nuoffer, Sabina Gallati, André Schaller
Isolated defects of the mitochondrial respiratory complex II (succinate dehydrogenase, SDH) are rare, accounting for approximately 2% of all respiratory chain deficiency diagnoses. Here, we report clinical and molecular investigations of three family members with a heterozygous mutation in the large flavoprotein subunit SDHA previously described to cause complex II deficiency. The index patient presented with bilateral optic atrophy and ocular movement disorder, a progressive polyneuropathy, psychiatric involvement, and cardiomyopathy...
September 28, 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27672511/bcl-2-homologue-debcl-enhances-%C3%AE-synuclein-induced-phenotypes-in-drosophila
#19
P Githure M'Angale, Brian E Staveley
BACKGROUND: Parkinson disease (PD) is a debilitating movement disorder that afflicts 1-2% of the population over 50 years of age. The common hallmark for both sporadic and familial forms of PD is mitochondrial dysfunction. Mammals have at least twenty proapoptotic and antiapoptotic Bcl-2 family members, in contrast, only two Bcl-2 family genes have been identified in Drosophila melanogaster, the proapoptotic mitochondrial localized Debcl and the antiapoptotic Buffy. The expression of the human transgene α-synuclein, a gene that is strongly associated with inherited forms of PD, in dopaminergic neurons (DA) of Drosophila, results in loss of neurons and locomotor dysfunction to model PD in flies...
2016: PeerJ
https://www.readbyqxmd.com/read/27664346/phylogeographic-structure-across-one-of-the-largest-intact-tropical-savannahs-molecular-and-morphological-analysis-of-australia-s-iconic-frilled-lizard-chlamydosaurus-kingii
#20
Mitzy Pepper, David G Hamilton, Thomas Merkling, Nina Svedin, Bori Cser, Renee A Catullo, Sarah R Pryke, J Scott Keogh
The spectacular threat display of the savannah specialist Australo-Papuan frilled lizards has made them one of the world's most iconic reptiles. They are increasingly used as a model system for research in evolutionary biology and ecology but little is known of their population structure. Their distribution across northern Australia and southern New Guinea also provides an opportunity to examine biogeographic patterns as they relate to the large-scale movement of savannah habitat during the Plio/Pleistocene and the associated increase in aridity...
September 21, 2016: Molecular Phylogenetics and Evolution
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