scholarly journals Serial cryoFIB/SEM reveals profound cytoarchitectural disruptions caused by a pathogenic mutation in Leigh syndrome patient cells

2020 ◽  
Author(s):  
Yanan Zhu ◽  
Dapeng Sun ◽  
Andreas Schertel ◽  
Jiying Ning ◽  
Xiaofeng Fu ◽  
...  
Keyword(s):  
Energy Production ◽  
Leigh Syndrome ◽  
Pathogenic Mutation ◽  
Syndrome Patient ◽  
Human Patient ◽  
Primary Fibroblasts ◽  
Mitochondrial Energy Production

AbstractThe advancement of serial cryo-FIB/SEM offers a new opportunity to study large volumes of near-native, fully hydrated frozen cells and tissues at voxel sizes of 10 nm and below. We explored this capability for pathologic characterization of vitrified human patient cells. We demonstrate profound disruption of subcellular architecture in primary fibroblasts from a Leigh syndrome patient harboring a disease-causing mutation in USMG5 protein responsible for impaired mitochondrial energy production.

Elaboration and Characterization of the Properties of Refractory Cr Base Alloys

2010 ◽  
Vol 72 ◽  
pp. 46-52 ◽  
Author(s):  
Laurent Royer ◽  
Stéphane Mathieu ◽  
Christophe Liebaut ◽  
Pierre Steinmetz
Keyword(s):  
Mechanical Properties ◽  
Melting Point ◽  
Molten Glass ◽  
Energy Production ◽  
Creep Properties ◽  
Refractory Alloys ◽  
Industrial Use ◽  
Kinetics Of ◽  
Selection Of

For energy production and also for the glass industry, finding new refractory alloys which could permit to increase the process temperatures to 1200°C or more is a permanent challenge. Chromium base alloys can be good candidates, considering the melting point of Cr itself, and also its low corrosion rate in molten glass. Two families of alloys have been studied for this purpose, Cr-Mo-W and Cr-Ta-X alloys (X= Mo, Si..). A finer selection of compositions has been done, to optimize their chemical and mechanical properties. Kinetics of HT oxidation by air, of corrosion by molten glass and also creep properties of several alloys have been measured up to 1250°C. The results obtained with the best alloys (Cr-Ta base) give positive indications as regards the possibility of their industrial use.

scholarly journals Scaling in a Geothermal Heat Exchanger at Soultz-Sous-Forêts (Upper Rhine Graben, France): A XRD and SEM-EDS Characterization of Sulfide Precipitates

Geosciences ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 271
Author(s):  
Béatrice A. Ledésert ◽  
Ronan L. Hébert ◽  
Justine Mouchot ◽  
Clio Bosia ◽  
Guillaume Ravier ◽  
...  
Keyword(s):  
Heat Exchanger ◽  
Energy Production ◽  
Electricity Production ◽  
Normal Operation ◽  
Upper Rhine Graben ◽  
Geothermal Heat ◽  
Deep Wells ◽  
Upper Rhine ◽  
Management Issues

The Soultz-Sous-Forêts geothermal site (France) operates three deep wells for electricity production. During operation, scales precipitate within the surface installation as (Ba, Sr) sulfate and (Pb, As, Sb) sulfide types. Scales have an impact on lowering energy production and inducing specific waste management issues. Thus scaling needs to be reduced for which a thorough characterization of the scales has to be performed. The geothermal brine is produced at 160 °C and reinjected at 70 °C during normal operation. In the frame of the H2020 MEET project, a small heat exchanger was tested in order to allow higher energy production, by reinjecting the geothermal fluid at 40 °C. Samples of scales were analyzed by XRD and SEM-EDS, highlighting that mostly galena precipitates and shows various crystal shapes. These shapes can be related to the turbulence of the flow and the speed of crystal growth. Where the flow is turbulent (entrance, water box, exit), crystals grow quickly and mainly show dendritic shape. In the tubes, where the flow is laminar, crystals grow more slowly and some of them are characterized by well-developed faces leading to cubes and derived shapes. The major consequence of the temperature decrease is the increased scaling phenomenon.

scholarly journals Generation and characterization of a human iPSC line SANi006-A from a Gray Platelet Syndrome patient

2021 ◽  
pp. 102443
Author(s):  
Cathelijn E.M. Aarts ◽  
Eszter Varga ◽  
Steven Webbers ◽  
Judy Geissler ◽  
Marieke von Lindern ◽  
...  
Keyword(s):  
Syndrome Patient ◽  
Ipsc Line ◽  
Gray Platelet Syndrome ◽  
Human Ipsc

scholarly journals Lower Mitochondrial Energy Production of the Thigh Muscles in Patients With Low‐Normal Ankle‐Brachial Index

2017 ◽  
Vol 6 (9) ◽  
Author(s):  
Majd AlGhatrif ◽  
Ariel Zane ◽  
Matt Oberdier ◽  
Marco Canepa ◽  
Stephanie Studenski ◽  
...  
Keyword(s):  
Energy Production ◽  
Ankle Brachial Index ◽  
Thigh Muscles ◽  
Mitochondrial Energy Production

Effect of mangiferin on mitochondrial energy production in experimentally induced myocardial infarcted rats

2006 ◽  
Vol 44 (6) ◽  
pp. 519-525 ◽  
Author(s):  
S. Prabhu ◽  
Mallika Jainu ◽  
K.E. Sabitha ◽  
C.S. Shyamala Devi
Keyword(s):  
Energy Production ◽  
Mitochondrial Energy Production ◽  
Experimentally Induced

scholarly journals Characterization of a 7.6-Mb germline deletion encompassing the NF1 locus and about a hundred genes in an NF1 contiguous gene syndrome patient

2008 ◽  
Vol 16 (12) ◽  
pp. 1459-1466 ◽  
Author(s):  
Eric Pasmant ◽  
Aurélie de Saint-Trivier ◽  
Ingrid Laurendeau ◽  
Anne Dieux-Coeslier ◽  
Béatrice Parfait ◽  
...  
Keyword(s):  
Syndrome Patient ◽  
Contiguous Gene

scholarly journals Methylpiperidinopyrazole Attenuates Estrogen-Induced Mitochondrial Energy Production and Subsequent Osteoblast Maturation via an Estrogen Receptor Alpha-Dependent Mechanism

Molecules ◽  
2020 ◽  
Vol 25 (12) ◽  
pp. 2876
Author(s):  
Poh-Shiow Yeh ◽  
Jui-Tai Chen ◽  
Yih-Giun Cherng ◽  
Shun-Tai Yang ◽  
Yu-Ting Tai ◽  
...  
Keyword(s):  
Energy Production ◽  
Estrogen Receptor Alpha ◽  
Type I Collagen ◽  
Atp Synthesis ◽  
Neonatal Rat ◽  
Type I ◽  
Collagen Mrna ◽  
Adenosine Phosphate ◽  
Mitochondrial Energy Production ◽  
Osteoblast Maturation

An estrogen deficiency is the main cause of osteoporosis in postmenopausal women. In bone remodeling, estrogen receptors (ERs) can mediate estrogen-transducing signals. Methylpiperidinopyrazole (MPP) is a highly specific antagonist of ER-alpha (ERα). This study was designed to evaluate the effects of MPP on estrogen-induced energy production, subsequent osteoblast maturation, and the possible mechanisms. Exposure of primary osteoblasts isolated from neonatal rat calvarias to MPP did not affect cell morphology or survival. Estradiol can induce translocation of ERα into mitochondria from the cytoplasm. Interestingly, pretreatment of rat calvarial osteoblasts with MPP lowered estrogen-induced ERα translocation. Sequentially, estrogen-triggered expressions of mitochondrial energy production-linked cytochrome c oxidase (COX) I and COX II messenger (m)RNAs were inhibited following pretreatment with MPP. Consequently, MPP caused decreases in estrogen-triggered augmentation of the activities of mitochondrial respiratory complex enzymes and levels of cellular adenosine phosphate (ATP). During progression of osteoblast maturation, estrogen induced bone morphogenetic protein (BMP)-6 and type I collagen mRNA expressions, but MPP treatment inhibited such induction. Consequently, estrogen-induced osteoblast activation and mineralization were attenuated after exposure to MPP. Taken together, MPP suppressed estrogen-induced osteoblast maturation through decreasing chromosomal osteogenesis-related BMP-6 and type I collagen mRNA expressions and mitochondrial ATP synthesis due to inhibiting energy production-linked COX I and II mRNA expressions. MPP can appropriately be applied to evaluate estrogen-involved bioenergetics and osteoblast maturation.

Hormonal regulation of mitochondrial energy production

2005 ◽  
Vol 8 (4) ◽  
pp. 415-418 ◽  
Author(s):  
Patrick Ritz ◽  
Jean-François Dumas ◽  
Pierre-Henri Ducluzeau ◽  
Gilles Simard
Keyword(s):  
Hormonal Regulation ◽  
Energy Production ◽  
Mitochondrial Energy Production
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