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Ronald J.A. Wanders,Gepke Visser,Sacha Ferdinandusse,Frédéric M. Vaz,Riekelt H. Houtkooper 한국지질동맥경화학회 2020 지질·동맥경화학회지 Vol.9 No.3
Mitochondrial fatty acid (FA) oxidation deficiencies represent a genetically heterogeneous group of diseases in humans caused by defects in mitochondrial FA beta-oxidation (mFAO). A general characteristic of all mFAO disorders is hypoketotic hypoglycemia resulting from the enhanced reliance on glucose oxidation and the inability to synthesize ketone bodies from FAs. Patients with a defect in the oxidation of long-chain FAs are at risk to develop cardiac and skeletal muscle abnormalities including cardiomyopathy and arrhythmias, which may progress into early death, as well as rhabdomyolysis and exercise intolerance. The diagnosis of mFAO-deficient patients has greatly been helped by revolutionary developments in the field of tandem mass spectrometry (MS) for the analysis of acylcarnitines in blood and/or urine of candidate patients. Indeed, acylcarnitines have turned out to be excellent biomarkers; not only do they provide information whether a certain patient is affected by a mFAO deficiency, but the acylcarnitine profile itself usually immediately points to which enzyme is likely deficient. Another important aspect of acylcarnitine analysis by tandem MS is that this technique allows high-throughput analysis, which explains why screening for mFAO deficiencies has now been introduced in many newborn screening programs worldwide. In this review, we will describe the current state of knowledge about mFAO deficiencies, with particular emphasis on recent developments in the area of pathophysiology and treatment.
Corrosion Fatigue of Austenitic Stainless Steel in Different Hot Chloride Solutions
( A. Visser ),( G. Mori ),( M. Panzenbock ),( R. Pippan ) 한국부식방식학회 2015 Corrosion Science and Technology Vol.14 No.4
Austenitic stainless steel was investigated under cyclic loading in electrolytes with different chloride contents and pH and at different temperatures. The testing solutions were 13.2 % NaCl (80,000 ppm Cl-) at 80 ¡ÆC and 43 % CaCl2 (275,000 ppm Cl-) at 120 ¡ÆC. In addition to S.N curves in inert and corrosive media, the fracture surfaces were investigated with a scanning electron microscope (SEM) to analyse the type of attack. The experimental results showed that a sharp decrease in corrosion fatigue properties can be correlated with the occurrence of stress corrosion cracking. The correlation of occurring types of damage in different corrosion systems is described.
Nurashikin Kemat,Richard G.F. Visser,Frans A. Krens The Korean Society of Plant Biotechnology 2023 JOURNAL OF PLANT BIOTECHNOLOGY Vol.50 No.1
Hyperhydricity is a physiological anomaly that significantly affects the growth and proliferation rate of crops cultivated by tissue culture techniques. To better understand the mechanisms that govern hyperhydricity incidence, we examined the effects of several media components, particularly cytokinin and gelling agents. These elements were found to be influential in both in vitro propagation and the development of hyperhydricity. Our study revealed that Arabidopsis thaliana seedlings had a greater manifestation of hyperhydricity symptoms when exposed to high cytokinin concentrations compared with the control. The presence of gelrite led to the manifestation of hyperhydric symptoms by elevated water build-up in the apoplast. The phenomenon of stomata closure was observed in the hyperhydric leaves, resulting in an increased ability to retain water and a decrease in the transpiration rates when compared to their respective control leaves. Additionally, histological examinations of the cross sections of hyperhydric leaves revealed an irregular cellular arrangement and large intercellular spaces. Furthermore, hyperhydric seedlings displayed impaired cuticular development in comparison to their normal seedlings.
Searching for Resonances in the Unbound 6Be Nucleus by Using a Radioactive 7Be Beam
채경육,D. W. Bardayan,J. C. Blackmon,M. S. Smith,A. E. Champagne,J. J. Das,R. P. Fitzgerald,D. W. Visser,V. Guimaraes,K. L. Jones,S. D. Pain,J. S. Thomas,M. S. Johnson,R. L. Kozub,R. J. Livesay,Z. Ma,C. 한국물리학회 2012 THE JOURNAL OF THE KOREAN PHYSICAL SOCIETY Vol.61 No.11
Knowledge of the <sup>3</sup>He(<sup>3</sup>He,<sup>2</sup><i>p</i>)<sup>4</sup>He reaction is important for understanding stellar burning and solar neutrino production. Previous measurements have found a surprisingly large rise in the cross section at low energies that could be due to a low-energy resonance in the <sup>3</sup>He + <sup>3</sup>He (<sup>6</sup>Be) system or electron screening. In the <sup>6</sup>Be nucleus, however, no excited states have been observed above the first 2<sup>+</sup> state at <i>E<sub>x</sub></i> = 1.67 MeV up to 23 MeV, even though several are expected. The <sup>2</sup>H(<sup>7</sup>Be,<sup>3</sup>H)<sup>6</sup>Be reaction has been studied for the first time to search for resonances in the <sup>6</sup>Be nucleus that may affect our understanding of the <sup>3</sup>He(<sup>3</sup>He,<sup>2</sup><i>p,</i>)<sup>4</sup>He reaction. A 100-MeV radioactive <sup>7</sup>Be beam from the Holifield Radioactive Ion Beam Facility (HRIBF) was used to bombard CD<sub>2</sub> targets, and tritons were detected by using the silicon detector array (SIDAR). A combination of reaction mechanisms appears to be necessary to explain the observed triton energy spectrum.
Lundberg, Ingrid E.,Tjä,rnlund, Anna,Bottai, Matteo,Werth, Victoria P.,Pilkington, Clarissa,de Visser, Marianne,Alfredsson, Lars,Amato, Anthony A.,Barohn, Richard J.,Liang, Matthew H.,Singh, Jasvi Wiley (John WileySons) 2017 Arthritis & Rheumatology Vol.69 No.12
<P>Conclusion. The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology, and pediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of 'definite,' 'probable,' and 'possible' IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria.</P>
Green, Joel D.,Evans II, Neal J.,Jørgensen, Jes K.,Herczeg, Gregory J.,Kristensen, Lars E.,Lee, Jeong-Eun,Dionatos, Odysseas,Yildiz, Umut A.,Salyk, Colette,Meeus, Gwendolyn,Bouwman, Jeroen,Visser, Ruu IOP Publishing 2013 The Astrophysical journal Vol.770 No.2
<P>We present 50-210 mu m spectral scans of 30 Class 0/I protostellar sources, obtained with Herschel-PACS, and 0.5-1000 mu m spectral energy distributions, as part of the Dust, Ice, and Gas in Time Key Program. Some sources exhibit up to 75 H2O lines ranging in excitation energy from 100 to 2000 K, 12 transitions of OH, and CO rotational lines ranging from J = 14 -> 13 up to J = 40 -> 39. [O I] is detected in all but one source in the entire sample; among the sources with detectable [O I] are two very low luminosity objects. The mean 63/145 mu m [O I] flux ratio is 17.2 +/- 9.2. The [O I] 63 mu m line correlates with L-bol, but not with the time-averaged outflow rate derived from low-J CO maps. [C II] emission is in general not local to the source. The sample L-bol increased by 1.25 (1.06) and T-bol decreased to 0.96 (0.96) of mean (median) values with the inclusion of the Herschel data. Most CO rotational diagrams are characterized by two optically thin components (< N > = ( 0.70 +/- 1.12) x 10(49) total particles). N-CO correlates strongly with L-bol, but neither T-rot nor N-CO(warm)/N-CO(hot) correlates with L-bol, suggesting that the total excited gas is related to the current source luminosity, but that the excitation is primarily determined by the physics of the interaction (e.g., UV-heating/shocks). Rotational temperatures for H2O (< T-rot > = 194 +/- 85 K) and OH (< T-rot > = 183 +/- 117 K) are generally lower than for CO, and much of the scatter in the observations about the best fit is attributed to differences in excitation conditions and optical depths among the detected lines.</P>