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https://www.readbyqxmd.com/read/29343244/hsp70-at-the-membrane-driving-protein-translocation
#1
REVIEW
Elizabeth A Craig
Efficient movement of proteins across membranes is required for cell health. The translocation process is particularly challenging when the channel in the membrane through which proteins must pass is narrow-such as those in the membranes of the endoplasmic reticulum and mitochondria. Hsp70 molecular chaperones play roles on both sides of these membranes, ensuring efficient translocation of proteins synthesized on cytosolic ribosomes into the interior of these organelles. The "import motor" in the mitochondrial matrix, which is essential for driving the movement of proteins across the mitochondrial inner membrane, is arguably the most complex Hsp70-based system in the cell...
January 17, 2018: BMC Biology
https://www.readbyqxmd.com/read/29341130/mitochondrial-function-and-autophagy-integrating-proteotoxic-redox-and-metabolic-stress-in-parkinson-s-disease
#2
REVIEW
Jianhua Zhang, M Lillian Culp, Jason G Craver, Victor Darley-Usmar
Parkinson's disease (PD) is a movement disorder with widespread neurodegeneration in the brain. Significant oxidative, reductive, metabolic, and proteotoxic alterations have been observed in PD postmortem brains. The alterations of mitochondrial function resulting in decreased bioenergetic health is important and needs to be further examined to help develop biomarkers for PD severity and prognosis. It is now becoming clear that multiple hits on metabolic and signaling pathways are likely to exacerbate PD pathogenesis...
January 17, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29321137/novel-function-of-ceramide-for-regulation-of-mitochondrial-atp-release-in-astrocytes
#3
Ji-Na Kong, Zhihui Zhu, Yutaka Itokazu, Guanghu Wang, Michael B Dinkins, Liansheng Zhong, Hsuan-Pei Lin, Ahmed Elsherbini, Silvia Leanhart, Xue Jiang, Haiyan Qin, Wenbo Zhi, Stefka D Spassieva, Erhard Bieberich
We reported that amyloid beta peptide (Abeta42) activated neutral sphingomyelinase 2 (nSMase2) thereby increasing the concentration of the sphingolipid ceramide in astrocytes. Here, we show that Abeta42 induced mitochondrial fragmentation in wild type astrocytes, but not in nSMase2-deficient cells or astrocytes treated with Fumonisin B1 (FB1), an inhibitor of ceramide synthases. Unexpectedly, ceramide depletion was concurrent with rapid movements of mitochondria, indicating an unknown function of ceramide for mitochondria...
January 10, 2018: Journal of Lipid Research
https://www.readbyqxmd.com/read/29320542/ancient-mitogenomes-of-phoenicians-from-sardinia-and-lebanon-a-story-of-settlement-integration-and-female-mobility
#4
E Matisoo-Smith, A L Gosling, D Platt, O Kardailsky, S Prost, S Cameron-Christie, C J Collins, J Boocock, Y Kurumilian, M Guirguis, R Pla Orquín, W Khalil, H Genz, G Abou Diwan, J Nassar, P Zalloua
The Phoenicians emerged in the Northern Levant around 1800 BCE and by the 9th century BCE had spread their culture across the Mediterranean Basin, establishing trading posts, and settlements in various European Mediterranean and North African locations. Despite their widespread influence, what is known of the Phoenicians comes from what was written about them by the Greeks and Egyptians. In this study, we investigate the extent of Phoenician integration with the Sardinian communities they settled. We present 14 new ancient mitogenome sequences from pre-Phoenician (~1800 BCE) and Phoenician (~700-400 BCE) samples from Lebanon (n = 4) and Sardinia (n = 10) and compare these with 87 new complete mitogenomes from modern Lebanese and 21 recently published pre-Phoenician ancient mitogenomes from Sardinia to investigate the population dynamics of the Phoenician (Punic) site of Monte Sirai, in southern Sardinia...
2018: PloS One
https://www.readbyqxmd.com/read/29307008/movement-disorders-in-mitochondrial-disease
#5
Roula Ghaoui, Carolyn M Sue
Mitochondrial disease presents with a wide spectrum of clinical manifestations that may appear at any age and cause multisystem dysfunction. A broad spectrum of movement disorders can manifest in mitochondrial diseases including ataxia, Parkinsonism, myoclonus, dystonia, choreoathetosis, spasticity, tremor, tic disorders and restless legs syndrome. There is marked heterogeneity of movement disorder phenotypes, even in patients with the same genetic mutation. Moreover, the advent of new technologies, such as next-generation sequencing, is likely to identify novel causative genes, expand the phenotype of known disease genes and improve the genetic diagnosis in these patients...
January 6, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29281717/genetic-origin-of-goat-populations-in-oman-revealed-by-mitochondrial-dna-analysis
#6
Nasser Ali Al-Araimi, Osman Mahgoub Gaafar, Vânia Costa, Agusto Luzuriaga Neira, Raed Mahmoud Al-Atiyat, Albano Beja-Pereira
The Sultanate of Oman has a complex mosaic of livestock species and production systems, but the genetic diversity, demographic history or origins of these Omani animals has not been expensively studied. Goats might constitute one of the most abundant and important domestic livestock species since the Neolithic transition. Here, we examined the genetic diversity, origin, population structure and demographic history of Omani goats. Specifically, we analyzed a 525-bp fragment of the first hypervariable region of the mitochondrial DNA (mtDNA) control region from 69 Omani individuals and compared this fragment with 17 mtDNA sequences from Somalia and Yemen as well as 18 wild goat species and 1,198 previously published goat sequences from neighboring countries...
2017: PloS One
https://www.readbyqxmd.com/read/29261068/expression-of-a-t39n-mutant-rab32-protein-arrests-mitochondria-movement-within-neurites-of-differentiated-sh-sy5y-cells
#7
Jonas Rybnicek, Samira Samtleben, Maria Sol Herrera-Cruz, Thomas Simmen
We have shown that multiple sclerosis (MS) and endoplasmic reticulum (ER) stress induce Rab32, an ER/mitochondria-localized small GTPase. High levels of both dominant-active (Q85L) or dominant-inactive (T39N) Rab32 are toxic to neurons. While Rab32Q85L interacts with its effector Drp1 to promote mitochondria fission, it is unclear how Rab32T39N could result as toxic to neurons. Given the perinuclear clustering of mitochondria observed upon transfection of inactive Rab32, we hypothesized Rab32T39N could stall mitochondria within neurites...
December 20, 2017: Small GTPases
https://www.readbyqxmd.com/read/29236757/high-genetic-structure-and-low-mitochondrial-diversity-in-bottlenose-dolphins-of-the-archipelago-of-bocas-del-toro-panama-a-population-at-risk
#8
Dalia C Barragán-Barrera, Laura J May-Collado, Gabriela Tezanos-Pinto, Valentina Islas-Villanueva, Camilo A Correa-Cárdenas, Susana Caballero
The current conservation status of the bottlenose dolphin (Tursiops truncatus) under the IUCN is 'least concern'. However, in the Caribbean, small and localized populations of the 'inshore form' may be at higher risk of extinction than the 'worldwide distributed form' due to a combination of factors including small population size, high site fidelity, genetic isolation, and range overlap with human activities. Here, we study the population genetic structure of bottlenose dolphins from the Archipelago of Bocas del Toro in Panama...
2017: PloS One
https://www.readbyqxmd.com/read/29230359/genetic-differentiation-and-phylogeography-of-mediterranean-north-eastern-atlantic-blue-shark-prionace-glauca-l-1758-using-mitochondrial-dna-panmixia-or-complex-stock-structure
#9
Agostino Leone, Ilenia Urso, Dimitrios Damalas, Jann Martinsohn, Antonella Zanzi, Stefano Mariani, Emilio Sperone, Primo Micarelli, Fulvio Garibaldi, Persefoni Megalofonou, Luca Bargelloni, Rafaella Franch, David Macias, Paulo Prodöhl, Séan Fitzpatrick, Marco Stagioni, Fausto Tinti, Alessia Cariani
Background: The blue shark (Prionace glauca, Linnaeus 1758) is one of the most abundant epipelagic shark inhabiting all the oceans except the poles, including the Mediterranean Sea, but its genetic structure has not been confirmed at basin and interoceanic distances. Past tagging programs in the Atlantic Ocean failed to find evidence of migration of blue sharks between the Mediterranean and the adjacent Atlantic, despite the extreme vagility of the species. Although the high rate of by-catch in the Mediterranean basin, to date no genetic study on Mediterranean blue shark was carried out, which constitutes a significant knowledge gap, considering that this population is classified as "Critically Endangered", unlike its open-ocean counterpart...
2017: PeerJ
https://www.readbyqxmd.com/read/29222186/new-splicing-variants-of-mitochondrial-rho-gtpase-1-miro1-transport-peroxisomes
#10
Kanji Okumoto, Tatsuaki Ono, Ryusuke Toyama, Ayako Shimomura, Aiko Nagata, Yukio Fujiki
Microtubule-dependent long-distance movement of peroxisomes occurs in mammalian cells. However, its molecular mechanisms remain undefined. In this study, we identified three distinct splicing variants of human mitochondrial Rho GTPase-1 (Miro1), each containing amino acid sequence insertions 1 (named Miro1-var2), 2 (Miro1-var3), and both 1 and 2 (Miro1-var4), respectively, at upstream of the transmembrane domain. Miro1-var4 and Miro1-var2 are localized to peroxisomes in a manner dependent on the insertion 1 that is recognized by the cytosolic receptor Pex19p...
December 8, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/29218863/population-structure-and-migration-of-the-tobacco-blue-mold-pathogen-peronospora-tabacina-into-north-america-and-europe
#11
Monica Blanco-Meneses, Ignazio Carbone, Jean B Ristaino
Tobacco blue mold, caused by Peronospora tabacina, is an oomycete plant pathogen that causes yearly epidemics in tobacco (Nicotiana tabacum) in the U.S. and Europe. The genetic structure of P. tabacina was examined in order to understand genetic diversity, population structure and patterns of migration. Two nuclear loci, Igs2 and Ypt1 and one mitochondrial locus, cox2, were amplified, cloned and sequenced from fifty four isolates of P. tabacina from the U.S., Central America-Caribbean-Mexico (CCAM), Europe and the Middle East (EULE)...
December 8, 2017: Molecular Ecology
https://www.readbyqxmd.com/read/29218782/therapy-development-in-huntington-disease-from-current-strategies-to-emerging-opportunities
#12
REVIEW
Audrey S Dickey, Albert R La Spada
Huntington disease (HD) is a progressive autosomal dominant neurodegenerative disorder in which patients typically present with uncontrolled involuntary movements and subsequent cognitive decline. In 1993, a CAG trinucleotide repeat expansion in the coding region of the huntingtin (HTT) gene was identified as the cause of this disorder. This extended CAG repeat results in production of HTT protein with an expanded polyglutamine tract, leading to pathogenic HTT protein conformers that are resistant to protein turnover, culminating in cellular toxicity and neurodegeneration...
December 8, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29211771/short-term-succinic-acid-treatment-mitigates-cerebellar-mitochondrial-oxphos-dysfunction-neurodegeneration-and-ataxia-in-a-purkinje-specific-spinocerebellar-ataxia-type-1-sca1-mouse-model
#13
Austin Ferro, Emily Carbone, Jenny Zhang, Evan Marzouk, Monica Villegas, Asher Siegel, Donna Nguyen, Thomas Possidente, Jessilyn Hartman, Kailen Polley, Melissa A Ingram, Georgia Berry, Thomas H Reynolds, Bernard Possidente, Kimberley Frederick, Stephen Ives, Sarita Lagalwar
Mitochondrial dysfunction plays a significant role in neurodegenerative disease including ataxias and other movement disorders, particularly those marked by progressive degeneration in the cerebellum. In this study, we investigate the role of mitochondrial oxidative phosphorylation (OXPHOS) deficits in cerebellar tissue of a Purkinje cell-driven spinocerebellar ataxia type 1 (SCA1) mouse. Using RNA sequencing transcriptomics, OXPHOS complex assembly analysis and oxygen consumption assays, we report that in the presence of mutant polyglutamine-expanded ataxin-1, SCA1 mice display deficits in cerebellar OXPHOS complex I (NADH-coenzyme Q oxidoreductase)...
2017: PloS One
https://www.readbyqxmd.com/read/29204154/mitochondria-a-common-target-for-genetic-mutations-and-environmental-toxicants-in-parkinson-s-disease
#14
REVIEW
Martin P Helley, Jennifer Pinnell, Carolina Sportelli, Kim Tieu
Parkinson's disease (PD) is a devastating neurological movement disorder. Since its first discovery 200 years ago, genetic and environmental factors have been identified to play a role in PD development and progression. Although genetic studies have been the predominant driving force in PD research over the last few decades, currently only a small fraction of PD cases can be directly linked to monogenic mutations. The remaining cases have been attributed to other risk associated genes, environmental exposures and gene-environment interactions, making PD a multifactorial disorder with a complex etiology...
2017: Frontiers in Genetics
https://www.readbyqxmd.com/read/29200367/anesthetic-management-in-mitochondrial-encephalomyopathy-a-case-report
#15
Toru Yamamoto, Noriko Miyazawa, Shinichi Yamamoto, Hiroshi Kawahara
We report on a morbidly obese 16-year-old boy (weight, 116 kg; height, 176 cm; body mass index, 35.5 kg/m2) with mitochondrial encephalomyopathy and a history of cerebral infarction, epilepsy, and severe mental retardation. The patient was scheduled for elective surgery under general anesthesia for multiple dental caries and entropion of the left eye. Preoperative examination results, including an electrocardiogram, were normal. No obvious cardiac function abnormalities were observed on echocardiography. Midazolam (10 mg) was administered orally as premedication 30 minutes before transfer to the operating room; however, the patient was uncooperative, and his body movements were difficult to control upon entering the operating room...
2017: Anesthesia Progress
https://www.readbyqxmd.com/read/29191733/trna-dynamics-between-the-nucleus-cytoplasm-and-mitochondrial-surface-location-location-location
#16
REVIEW
Kunal Chatterjee, Regina T Nostramo, Yao Wan, Anita K Hopper
Although tRNAs participate in the essential function of protein translation in the cytoplasm, tRNA transcription and numerous processing steps occur in the nucleus. This subcellular separation between tRNA biogenesis and function requires that tRNAs be efficiently delivered to the cytoplasm in a step termed "primary tRNA nuclear export". Surprisingly, tRNA nuclear-cytoplasmic traffic is not unidirectional, but, rather, movement is bidirectional. Cytoplasmic tRNAs are imported back to the nucleus by the "tRNA retrograde nuclear import" step which is conserved from budding yeast to vertebrate cells and has been hijacked by viruses, such as HIV, for nuclear import of the viral reverse transcription complex in human cells...
November 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29187824/the-implication-of-pgc-1%C3%AE-on-fatty-acid-transport-across-plasma-and-mitochondrial-membranes-in-the-insulin-sensitive-tissues
#17
REVIEW
Elżbieta Supruniuk, Agnieszka Mikłosz, Adrian Chabowski
PGC-1α coactivator plays a decisive role in the maintenance of lipid balance via engagement in numerous metabolic processes (i.e., Krebs cycle, β-oxidation, oxidative phosphorylation and electron transport chain). It constitutes a link between fatty acids import and their complete oxidation or conversion into bioactive fractions through the coordination of both the expression and subcellular relocation of the proteins involved in fatty acid transmembrane movement. Studies on cell lines and/or animal models highlighted the existence of an upregulation of the total and mitochondrial FAT/CD36, FABPpm and FATPs content in skeletal muscle in response to PGC-1α stimulation...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29177768/familial-parkinson-s-disease-associated-l166p-mutant-dj-1-is-cleaved-by-mitochondrial-serine-protease-omi-htra2
#18
Kai Fu, Yanfei Wang, Dongkai Guo, Guanghui Wang, Haigang Ren
Parkinson's disease (PD) is the most common neurodegenerative movement disorder. Mutations in the DJ-1, including L166P, are responsible for recessive early-onset PD. Many lines of evidence have shown that L166P is not only a loss-of-function mutant, but also a pro-apoptotic-like protein that results in mitochondrial dysfunction. L166P has been reported to be unstable and to mislocalize to mitochondria. However, the mechanisms underlying the instability of L166P compared to wild-type DJ-1 remain largely unknown...
November 24, 2017: Neuroscience Bulletin
https://www.readbyqxmd.com/read/29175325/fatty-acid-oxidation-alleviates-the-energy-deficiency-caused-by-the-loss-of-mpc1-in-mpc1-mice
#19
Shiying Zou, Tianqi Lang, Boyang Zhang, Kunlun Huang, Lijing Gong, Haosu Luo, Wentao Xu, Xiaoyun He
Pyruvate is a central substrate in energy metabolism, paramount to carbohydrate, fat, and amino acid catabolic and anabolic pathways. Mitochondrial pyruvate carrier 1(MPC1) is one important component of the complex that facilitates mitochondrial pyruvate import. Complete MPC1 deficiency is a serious concern, and has been shown to result in embryonic lethality in mice. The study outlined in this paper generated one mouse line with the MPC1 protein part deficiency by using the CRISPR/Cas9 system. Clinical observations, body weight and organ/tissue weight, gas exchange, cold-stimulation, blood parameters, as well as histopathology analysis were analyzed to evaluate potential physiological abnormalities caused by MPC1 deficiency...
November 21, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29167359/genome-diversity-in-the-neolithic-globular-amphorae-culture-and-the-spread-of-indo-european-languages
#20
Francesca Tassi, Stefania Vai, Silvia Ghirotto, Martina Lari, Alessandra Modi, Elena Pilli, Andrea Brunelli, Roberta Rosa Susca, Alicja Budnik, Damian Labuda, Federica Alberti, Carles Lalueza-Fox, David Reich, David Caramelli, Guido Barbujani
It is unclear whether Indo-European languages in Europe spread from the Pontic steppes in the late Neolithic, or from Anatolia in the Early Neolithic. Under the former hypothesis, people of the Globular Amphorae culture (GAC) would be descended from Eastern ancestors, likely representing the Yamnaya culture. However, nuclear (six individuals typed for 597 573 SNPs) and mitochondrial (11 complete sequences) DNA from the GAC appear closer to those of earlier Neolithic groups than to the DNA of all other populations related to the Pontic steppe migration...
November 29, 2017: Proceedings. Biological Sciences
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