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https://www.readbyqxmd.com/read/29615062/kars-related-diseases-progressive-leukoencephalopathy-with-brainstem-and-spinal-cord-calcifications-as-new-phenotype-and-a-review-of-literature
#1
Anna Ardissone, Davide Tonduti, Andrea Legati, Eleonora Lamantea, Rita Barone, Imen Dorboz, Odile Boespflug-Tanguy, Gabriella Nebbia, Marco Maggioni, Barbara Garavaglia, Isabella Moroni, Laura Farina, Anna Pichiecchio, Simona Orcesi, Luisa Chiapparini, Daniele Ghezzi
BACKGROUND: KARS encodes lysyl- transfer ribonucleic acid (tRNA) synthetase, which catalyzes the aminoacylation of tRNA-Lys in the cytoplasm and mitochondria. Eleven families/sporadic patients and 16 different mutations in KARS have been reported to date. The associated clinical phenotype is heterogeneous ranging from early onset encephalopathy to isolated peripheral neuropathy or nonsyndromic hearing impairment. Recently additional presentations including leukoencephalopathy as predominant cerebral involvement or cardiomyopathy, isolated or associated with muscular and cerebral involvement, have been reported...
April 4, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29563994/shikonin-induces-mitochondria-mediated-apoptosis-and-attenuates-epithelial-mesenchymal-transition-in-cisplatin-resistant-human-ovarian-cancer-cells
#2
Kristina Shilnikova, Mei Jing Piao, Kyoung Ah Kang, Yea Seong Ryu, Jeong Eon Park, Yu Jae Hyun, Ao Xuan Zhen, Yong Joo Jeong, Uhee Jung, In Gyu Kim, Jin Won Hyun
Cisplatin-based chemotherapy often results in the development of chemoresistance when used to treat ovarian cancer, which is difficult to overcome. The present study investigated the cytotoxic and anti-migratory effects of shikonin, a naphthoquinone compound, on cisplatin-resistant human ovarian cancer A2780 cells (A2780-CR). Shikonin had a potent dose-dependent cytotoxic effect on A2780-CR cells, with 9 µM shikonin treatment reducing A2780-CR cell viability by 50%, validate using an MTT assay. Shikonin induced apoptosis, as evidenced by the increased number of apoptotic bodies, following staining with Hoechst 33342, and terminal deoxynucleotidyl cell transferase dUTP nick end labeling-positive cells following treatment...
April 2018: Oncology Letters
https://www.readbyqxmd.com/read/29562677/miro1-enhances-mitochondria-transfer-from-multipotent-mesenchymal-stem-cells-mmsc-to-neural-cells-and-improves-the-efficacy-of-cell-recovery
#3
Valentina A Babenko, Denis N Silachev, Vasily A Popkov, Ljubava D Zorova, Irina B Pevzner, Egor Y Plotnikov, Gennady T Sukhikh, Dmitry B Zorov
A recently discovered key role of reactive oxygen species (ROS) in mitochondrial traffic has opened a wide alley for studying the interactions between cells, including stem cells. Since its discovery in 2006, intercellular mitochondria transport has been intensively studied in different cellular models as a basis for cell therapy, since the potential of replacing malfunctioning organelles appears to be very promising. In this study, we explored the transfer of mitochondria from multipotent mesenchymal stem cells (MMSC) to neural cells and analyzed its efficacy under normal conditions and upon induction of mitochondrial damage...
March 19, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29531533/metabolic-heterogeneity-evidenced-by-mrs-among-patient-derived-glioblastoma-multiforme-stem-like-cells-accounts-for-cell-clustering-and-different-responses-to-drugs
#4
Sveva Grande, Alessandra Palma, Lucia Ricci-Vitiani, Anna Maria Luciani, Mariachiara Buccarelli, Mauro Biffoni, Agnese Molinari, Annarica Calcabrini, Emanuela D'Amore, Laura Guidoni, Roberto Pallini, Vincenza Viti, Antonella Rosi
Clustering of patient-derived glioma stem-like cells (GSCs) through unsupervised analysis of metabolites detected by magnetic resonance spectroscopy (MRS) evidenced three subgroups, namely clusters 1a and 1b, with high intergroup similarity and neural fingerprints, and cluster 2, with a metabolism typical of commercial tumor lines. In addition, subclones generated by the same GSC line showed different metabolic phenotypes. Aerobic glycolysis prevailed in cluster 2 cells as demonstrated by higher lactate production compared to cluster 1 cells...
2018: Stem Cells International
https://www.readbyqxmd.com/read/29513101/rip140-pgc-1%C3%AE-axis-involved-in-vitamin-a-induced-neural-differentiation-by-increasing-mitochondrial-function
#5
Qing Mu, Weidong Yu, Shuying Zheng, Hongxia Shi, Mei Li, Jie Sun, Di Wang, Xiaoli Hou, Ling Liu, Xinjuan Wang, Zhuran Zhao, Rong Liang, Xue Zhang, Wei Dong, Chaomei Zeng, Jingzhu Guo
Vitamin A deficiency and mitochondrial dysfunction are both associated with neural differentiation-related disorders, such as Alzheimer's disease (AD) and Down syndrome (DS). The mechanism of vitamin A-induced neural differentiation and the notion that vitamin A can regulate the morphology and function of mitochondria in its induction of neural differentiation through the RIP140/PGC-1α axis are unclear. The aim of this study was to investigate the roles and underlying mechanisms of RIP140/PGC-1α axis in vitamin A-induced neural differentiation...
March 7, 2018: Artificial Cells, Nanomedicine, and Biotechnology
https://www.readbyqxmd.com/read/29496782/brain-mitochondrial-bioenergetics-change-with-rapid-and-prolonged-shifts-in-aggression-in-the-honey-bee-apis-mellifera
#6
Clare C Rittschof, Hemendra J Vekaria, Joseph H Palmer, Patrick G Sullivan
Neuronal function demands high-level energy production, and as such, a decline in mitochondrial respiration characterizes brain injury and disease. A growing number of studies, however, link brain mitochondrial function to behavioral modulation in non-diseased contexts. In the honey bee, we show for the first time that an acute social interaction, which invokes an aggressive response, may also cause a rapid decline in brain mitochondrial bioenergetics. The degree and speed of this decline has only been previously observed in the context of brain injury...
March 1, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29495951/mitochondrial-dysfunction-maternal-protein-restriction-as-a-trigger-of-reactive-species-overproduction-and-brainstem-energy-failure-in-male-offspring-brainstem
#7
D J S Ferreira, A A Pedroza, G R F Braz, M P Fernandes, C J Lagranha
Mitochondria are important organelles in eukaryotic organisms, wherein their capacity to produce energy vary among the tissues depending upon the amounts of oxygen consumed. Part of the oxygen consumed during ATP generation produces reactive oxygen species, which if not efficiently removed can trigger a systemic damage to molecular compounds characterized as oxidative stress. Several studies have demonstrated that mitochondrial dysfunction and oxidative stress in the central nervous system (CNS) are related to a plethora of neural disorders...
March 1, 2018: Nutritional Neuroscience
https://www.readbyqxmd.com/read/29466734/pharmacological-inhibition-of-necroptosis-protects-from-dopaminergic-neuronal-cell-death-in-parkinson-s-disease-models
#8
Angelo Iannielli, Simone Bido, Lucrezia Folladori, Alice Segnali, Cinzia Cancellieri, Alessandra Maresca, Luca Massimino, Alicia Rubio, Giuseppe Morabito, Leonardo Caporali, Francesca Tagliavini, Olimpia Musumeci, Giuliana Gregato, Erwan Bezard, Valerio Carelli, Valeria Tiranti, Vania Broccoli
Dysfunctions in mitochondrial dynamics and metabolism are common pathological processes associated with Parkinson's disease (PD). It was recently shown that an inherited form of PD and dementia is caused by mutations in the OPA1 gene, which encodes for a key player in mitochondrial fusion and structure. iPSC-derived neural cells from these patients exhibited severe mitochondrial fragmentation, respiration impairment, ATP deficits, and heightened oxidative stress. Reconstitution of normal levels of OPA1 in PD-derived neural cells normalized mitochondria morphology and function...
February 20, 2018: Cell Reports
https://www.readbyqxmd.com/read/29453779/mitochondrial-cytochrome-p450-cyp-1b1-is-responsible-for-melatonin-induced-apoptosis-in-neural-cancer-cells
#9
Zhenlong Yu, Xiangge Tian, Yuling Peng, Zheng Sun, Chao Wang, Ning Tang, Bin Li, Yuqing Jian, Wei Wang, Xiaokui Huo, Xiaochi Ma
Melatonin is an endogenous indoleamine with a wide range of biological functions in the various organisms from bacteria to mammals. Evidence indicates that melatonin facilitates apoptosis in cancer cells and enhances the anti-tumor activity of chemotherapy in animals and clinical studies. However, the melatonin metabolism and the key metabolic targets in cancer cells still remain unknown. In this study, U118 and SH-SY5Y tumor cell lines were used to investigate the metabolic pathways of melatonin in cancer cells...
February 17, 2018: Journal of Pineal Research
https://www.readbyqxmd.com/read/29445135/alteration-in-nerves-and-neurotransmitter-stimulation-of-lacrimal-gland-secretion-in-the-tsp-1-mouse-model-of-aqueous-deficiency-dry-eye
#10
Sumit Bhattacharya, Laura García-Posadas, Robin R Hodges, Helen P Makarenkova, Sharmila Masli, Darlene A Dartt
The purpose of this study is to determine neural, vascular, protein secretion, and cellular signaling changes with disease progression in lacrimal glands of the thrombospondin-1 -/- (TSP-1 -/- ) mouse model of dry eye compared to C57BL/6 wild-type (WT) mice. Neural innervation was reduced in TSP-1 -/- lacrimal glands compared to WT controls, whereas the number of blood vessels was increased. Intracellular Ca 2+ stores and the amount of lysosomes, mitochondria, and secretory granules, but not the endoplasmic reticulum, were reduced in TSP-1 -/- compared to WT acini at 12 weeks of age...
February 14, 2018: Mucosal Immunology
https://www.readbyqxmd.com/read/29441716/behavioural-mating-displays-depend-on-mitochondrial-function-a-potential-mechanism-for-linking-behaviour-to-individual-condition
#11
Rebecca E Koch, Geoffrey E Hill
Males of many animal species court females using complex behavioural displays that are challenging to produce, and some of these displays have been shown to be associated with aspects of male quality. However, the mechanisms by which behavioural displays are linked to individual condition remain uncertain. Herein, we illustrate fundamental mechanistic connections between mitochondrial function and neurogenesis, energy production, and a variety of pathways that underlie the ability of an individual to perform complex behaviours...
February 13, 2018: Biological Reviews of the Cambridge Philosophical Society
https://www.readbyqxmd.com/read/29419416/als-lymphoblastoid-cell-lines-as-a-considerable-model-to-understand-disease-mechanisms
#12
O Pansarasa, M Bordoni, L Dufruca, L Diamanti, D Sproviero, R Trotti, S Bernuzzi, S La Salvia, S Gagliardi, M Ceroni, C Cereda
New evidences switch the hypothesis of amyotrophic lateral sclerosis (ALS) from a "neurocentric" to a "multisystemic" or "non-neurocentric" point of view. From 2006, we focused on the study of non-neural cells, patients' peripheral blood mononuclear cells (PMBCs) and lymphoblastoid cell lines (LCLs). Here, we characterized LCLs of sporadic ALS and patients carrying SOD1, TARDBP and FUS mutations to identify ALS biologically relevant signature, and whether and how mutations differentially affect ALS-linked pathways...
January 29, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29416846/pml-regulation-and-multifaceted-function-beyond-tumor-suppression
#13
REVIEW
Kuo-Sheng Hsu, Hung-Ying Kao
Promyelocytic leukemia protein (PML) was originally identified as a fusion partner of retinoic acid receptor alpha in acute promyelocytic leukemia patients with the (15;17) chromosomal translocation, giving rise to PML-RARα and RARα-PML fusion proteins. A body of evidence indicated that PML possesses tumor suppressing activity by regulating apoptosis, cell cycle, senescence and DNA damage responses. PML is enriched in discrete nuclear substructures in mammalian cells with 0.2-1 μm diameter in size, referred to as alternately Kremer bodies, nuclear domain 10, PML oncogenic domains or PML nuclear bodies (NBs)...
2018: Cell & Bioscience
https://www.readbyqxmd.com/read/29413961/13-reasons-why-the-brain-is-susceptible-to-oxidative-stress
#14
REVIEW
James Nathan Cobley, Maria Luisa Fiorello, Damian Miles Bailey
The human brain consumes 20% of the total basal oxygen (O2 ) budget to support ATP intensive neuronal activity. Without sufficient O2 to support ATP demands, neuronal activity fails, such that, even transient ischemia is neurodegenerative. While the essentiality of O2 to brain function is clear, how oxidative stress causes neurodegeneration is ambiguous. Ambiguity exists because many of the reasons why the brain is susceptible to oxidative stress remain obscure. Many are erroneously understood as the deleterious result of adventitious O2 derived free radical and non-radical species generation...
May 2018: Redox Biology
https://www.readbyqxmd.com/read/29395479/insufficient-apaf-1-expression-in-early-stages-of-neural-differentiation-of-human-embryonic-stem-cells-might-protect-them-from-apoptosis
#15
Somayeh Karimzadeh, Saman Hosseinkhani, Ali Fathi, Farangis Ataei, Hossein Baharvand
Recent evidence suggests that mitochondrial apoptosis regulators and executioners may regulate differentiation, without being involved in cell death. However, the involved factors and their roles in differentiation and apoptosis are still not fully determined. In the present study, we compared mitochondrial pathway of cell death during early neural differentiation from human embryonic stem cells (hESCs). Our results demonstrated that ROS generation, cytosolic cytochrome c release, caspases activation and rise in p53 protein level occurred upon either neural or apoptosis induction in hESCs...
January 30, 2018: European Journal of Cell Biology
https://www.readbyqxmd.com/read/29362000/determining-direction-of-axonal-flow-in-the-equine-ramus-communicans-by-ultrastructural-examination-of-the-plantar-nerves-2-months-after-transecting-the-ramus
#16
Fakhri Al-Bagdadi, Jim Schumacher, Jessi Carter, Ferenc Tóth, Robert W Henry
The ramus communicans, neural connection between medial and lateral plantar nerves of the horse, was transected to determine the degree to which medial and lateral plantar nerves contribute to the plantar ramus. After 2 months, sections of plantar nerves immediately proximal and distal to the communicating branch were collected and processed for electron microscopy. All examined nerves had undergone Wallerian degeneration and contained regenerating and mature fibers. Layers of the myelin sheath were separated by spaces and vacuoles, indicating demyelination of medial and lateral plantar nerves...
January 24, 2018: Microscopy and Microanalysis
https://www.readbyqxmd.com/read/29353044/bmp4-signaling-in-npcs-upregulates-bcl-xl-to-promote-their-survival-in-the-presence-of-fgf-2
#17
Hanako Yamamoto, Masashi Kurachi, Masae Naruse, Koji Shibasaki, Yasuki Ishizaki
We previously reported that BMP4 does not promote proliferation or differentiation of CD44-positive astrocyte precursor cells (APCs) but greatly promotes their survival in the presence of fibroblast growth factor-2 (FGF-2). In this study, we examined if BMP4 acts as a survival factor also for neural stem/progenitor cells (NPCs) isolated from ganglionic eminence of neonatal mouse brain. We found BMP4 promotes survival but not proliferation or differentiation of these cells, just as in the case for CD44-positive APCs...
January 15, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29339091/an-energetic-view-of-stress-focus-on-mitochondria
#18
REVIEW
Martin Picard, Bruce S McEwen, Elissa S Epel, Carmen Sandi
Energy is required to sustain life and enable stress adaptation. At the cellular level, energy is largely derived from mitochondria - unique multifunctional organelles with their own genome. Four main elements connect mitochondria to stress: (1) Energy is required at the molecular, (epi)genetic, cellular, organellar, and systemic levels to sustain components of stress responses; (2) Glucocorticoids and other steroid hormones are produced and metabolized by mitochondria; (3) Reciprocally, mitochondria respond to neuroendocrine and metabolic stress mediators; and (4) Experimentally manipulating mitochondrial functions alters physiological and behavioral responses to psychological stress...
January 12, 2018: Frontiers in Neuroendocrinology
https://www.readbyqxmd.com/read/29335845/carnosic-acid-as-a-promising-agent-in-protecting-mitochondria-of-brain-cells
#19
REVIEW
Marcos Roberto de Oliveira
Carnosic acid (CA; C20 H28 O4 ), a phenolic diterpene characterized as an ortho-dihydroquinone-type molecule, is a pro-electrophile agent that becomes an electrophile after reacting with free radicals. The electrophile generated from CA interacts with and activates the nuclear factor erythroid 2-related factor 2 (Nrf2) transcription factor, which is a major modulator of redox biology in mammalian cells. CA induces antioxidant and anti-inflammatory effects in several cell types, as observed in both in vitro and in vivo experimental models...
January 15, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29284498/accelerated-super-resolution-imaging-with-fret-paint
#20
Jongjin Lee, Sangjun Park, Wooyoung Kang, Sungchul Hohng
Super-resolution fluorescence microscopy in the current form is hard to be used to image the neural connectivity of thick tissue samples due to problems such as slow imaging speed, severe photobleaching of fluorescent probes, and high background noise. Recently developed DNA-PAINT solved the photobleaching problem, but its imaging speed is extremely low. We report accelerated super-resolution fluorescence microscopy named FRET-PAINT. Compared to conventional DNA-PAINT, the imaging speed of the microscopy increases up to ~30-fold...
December 28, 2017: Molecular Brain
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