Article
Biochemistry & Molecular Biology
Ravi R. Sonani, Artur Blat, Grzegorz Dubin
Summary: This study solved the crystal structures of human ACOX1a, revealing its homodimeric form and the binding of two FAD molecules at the active site. It also found that the substrate binding cleft of ACOX1a is wider compared to other fatty acid oxidation enzymes. Furthermore, the study analyzed the impact of mutations causing dysfunctionality of ACOX1a on its structure and function.
INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES
(2022)
Article
Gastroenterology & Hepatology
Mary P. Moore, Rory P. Cunningham, Rachel A. H. Davis, Sarah E. Deemer, Brandon M. Roberts, Eric P. Plaisance, R. Scott Rector
Summary: This study showed that dietary administration of the ketone ester BD-AcAc2 attenuates hepatic steatosis, inflammation, fibrosis, and improves liver health markers in mice with HFD-induced NAFLD. The results suggest potential therapeutic benefits of the ketone ester in treating metabolic diseases like NAFLD.
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY
(2021)
Article
Multidisciplinary Sciences
Anastasia Lisuzzo, Luca Laghi, Vanessa Faillace, Chenglin Zhu, Barbara Contiero, Massimo Morgante, Elisa Mazzotta, Matteo Gianesella, Enrico Fiore
Summary: The mobilization of body reserves during the transition from pregnancy to lactation may lead to metabolic disorders in dairy cows. This study evaluated the differences in serum metabolome composition based on beta-hydroxybutyrate (BHB) concentration, finding metabolites related to body reserve mobilization, lipid metabolism, amino acid and carbohydrate metabolism, and ruminal fermentation.
SCIENTIFIC REPORTS
(2022)
Article
Biochemistry & Molecular Biology
Rasmus Stenlid, Hannes Manell, Rikard Seth, Sara Y. Cerenius, Azazul Chowdhury, Camilla Roa Cortes, Isabelle Nyqvist, Thomas Lundqvist, Maria Halldin, Peter Bergsten
Summary: This study found that patients with VLCAD and MCAD have significantly lower plasma glucagon concentrations compared to patients with CUD, while the concentrations in the CUD group were similar to normative data, suggesting that glucagon secretion may be impaired in patients with VLCAD or MCAD.
Article
Biochemistry & Molecular Biology
Laurent Suissa, Pavel Kotchetkov, Jean-Marie Guigonis, Emilie Doche, Ophelie Osman, Thierry Pourcher, Sabine Lindenthal
Summary: The role of ketone bodies in cerebral energy homeostasis has attracted attention in acute neurological diseases. Oral ingestion of ketone ester leads to increased acetyl-CoA and citric cycle intermediates in the brain, inhibiting glycolysis and competing with glucose.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Editorial Material
Clinical Neurology
Brandon B. Holmes, Jeffrey B. Russ, Yvonne W. Wu, Renata C. Gallagher, Dawn Gano
Summary: The newborn male infant was born prematurely at 36 weeks, with low birth weight, but he behaved normally and breastfed well in the first 40 hours of life.
Article
Biotechnology & Applied Microbiology
Chenyang Yuan, Yixin Li, Suwan Han, Beiru He, Xinghui Zhai, Weichao Lin, Jiping Shi, Junsong Sun, Baoguo Zhang
Summary: This study identified and studied three ChsE enzymes in Mycolicibacterium neoaurum, comparing their functions with those from Mycobacterium tuberculosis. The results showed that ChsE4-ChsE5 could metabolize C27, C24, and C22 steroids in vivo, ChsE3 could metabolize C27 and C24 steroids in vivo, and ChsE1-ChsE2 could metabolize C27, C24, and C22 steroids in vivo. Additionally, the overexpression of ChsE led to the production of a new C22 steroid, 3-oxo-4,17-pregadiene-20-carboxylic acid methyl ester (PDCE). This study enhanced the understanding of steroid bioconversion pathways and provided a method for the production of novel C22 steroids.
APPLIED MICROBIOLOGY AND BIOTECHNOLOGY
(2023)
Article
Clinical Neurology
Hideaki Shiraishi, Kenji Yamada, Kiyoshi Egawa, Mika Ishige, Fumihiro Ochi, Asami Watanabe, Sanae Kawakami, Kazuyo Kuzume, Kenji Watanabe, Koji Sameshima, Kiyotaka Nakamagoe, Akira Tamaoka, Naoko Asahina, Saki Yokoshiki, Keiko Kobayashi, Takashi Miyakoshi, Koji Oba, Toshiyuki Isoe, Hiroshi Hayashi, Seiji Yamaguchi, Norihiro Sato
Summary: The study demonstrated the potential long-term efficacy of bezafibrate in preventing myopathic attacks for patients with VLCADD, significantly reducing the number of attacks requiring treatment and improving patients' ability to conduct everyday activities.
BRAIN & DEVELOPMENT
(2021)
Article
Pediatrics
Tracy L. McGregor, Susan A. Berry, Katrina M. Dipple, Rizwan Hamid
Summary: Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCADD) is a fatty acid oxidation disorder where patients cannot break down fats for energy. Pediatricians should provide acute clinical care for patients with MCADD and work with primary metabolic physicians to prevent adverse outcomes for children.
Article
Multidisciplinary Sciences
Yuxun Zhang, Eric Goetzman
Summary: Mitochondrial trifunctional protein (TFP) plays a crucial role in chain-shortening long-chain fatty acids within the mitochondria. Despite being heavily modified by acetyllysine and succinyllysine post-translational modifications, the functional significance of these modifications on TFP remains unclear. Research suggests that TFP in the membrane-bound state may be protected from lysine acylation, which could impact its enzymatic activity.
Article
Chemistry, Organic
Samson Lai, Noah Takaesu, Wen Xuan Lin, David M. Perrin
Summary: Recent studies have investigated the potential use of acyl-MIDA boronate esters as substrates in the Suzuki-Miyaura cross-coupling reaction. It was found that benzoyl-MIDA boronate can successfully undergo C-C cross-coupling with various aryl bromide substrates to produce benzophenones in adequate yields under optimized conditions. However, other acyl-MIDA boronate esters in an alkyl series were not suitable coupling partners, limiting the substrate scope.
TETRAHEDRON LETTERS
(2021)
Article
Biotechnology & Applied Microbiology
Tao Wang, Hao Shi, William B. Whitman
Summary: DmdC1 from R. pomeroyi shows high affinity for short-chain acyl-CoAs in addition to its natural substrate MMPA-CoA, and is not affected by potential effectors tested, suggesting minimal adaptations to DMSP metabolism and recent evolution from a short-chain acyl-CoA dehydrogenase involved in fatty acid oxidation.
APPLIED AND ENVIRONMENTAL MICROBIOLOGY
(2022)
Article
Endocrinology & Metabolism
Willemijn J. van Rijt, Johan L. K. Van Hove, Frederic M. Vaz, Rick Havinga, Derk P. Allersma, Tanja R. Zijp, Jirair K. Bedoyan, M. R. Heiner-Fokkema, Dirk-Jan Reijngoud, Michael T. Geraghty, Ronald J. A. Wanders, Maaike H. Oosterveer, Terry G. J. Derks
Summary: Our study investigated the enantiomer-specific pharmacokinetics of D,L-3-hydroxybutyrate (D,L-3-HB) and found that treatment with D,L-3-HB resulted in significantly higher concentrations of L-3-HB. Both D-3-HB and L-3-HB peaked at 30-60 minutes after administration in MADD patients and rats, with a gradual return to baseline levels after 3 hours. Additionally, the distribution of D,L-3-HB led to differential metabolic fates of D-3-HB and L-3-HB in various tissues.
JOURNAL OF INHERITED METABOLIC DISEASE
(2021)
Article
Food Science & Technology
L. T. Claire M. Modica, Krystal Flores-Felix, L. T. John D. Casachahua, Paul Asquith, Anna Tschiffely, Stephanie Ciarlone, Stephen T. Ahlers
Summary: Both ketogenic diet and ketone ester supplements increase ketone levels and decrease weight compared to a standard diet. Ketogenic diet has a stronger effect on glucose levels, while sex differences play a role in weight changes.
FOOD CHEMISTRY: MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Zhesi Zhu, Zhen Han, Levon Halabelian, Xiangkun Yang, Jun Ding, Nawei Zhang, Liza Ngo, Jiabao Song, Hong Zeng, Maomao He, Yingming Zhao, Cheryl H. Arrowsmith, Minkui Luo, Michael G. Bartlett, Y. George Zheng
Summary: Lysine isobutyrylation (Kibu) is a newly discovered posttranslational chemical modification on nuclear histones, derived from isobutyryl-CoA. Various histone acetyltransferases exhibit lysine isobutyryltransferase activity in vitro, with p300 being a major regulator of histone isobutyrylation levels in cells. The X-ray crystal structures of HAT1 in complex with isobutyryl-CoA provide insight into HAT-catalyzed isobutyrylation, and RNA-Seq profiling shows that isobutyrate greatly affects gene expression in pivotal biological pathways.
NUCLEIC ACIDS RESEARCH
(2021)
Article
Endocrinology & Metabolism
Monique Albersen, Samantha L. van der Beek, Inge M. E. Dijkstra, Marielle Alders, Rinse W. Barendsen, Jet Bliek, Anita Boelen, Merel S. Ebberink, Sacha Ferdinandusse, Susan M. Goorden, Annemieke C. Heijboer, Mandy Jansen, Yorrick R. J. Jaspers, Ingrid Metgod, Gajja S. Salomons, Frederic M. Vaz, Rendelien K. Verschoof-Puite, Wouter F. Visser, Eugenie Dekkers, Marc Engelen, Stephan Kemp
Summary: Males with X-linked adrenoleukodystrophy (ALD) are at high risk for developing adrenal insufficiency and/or progressive leukodystrophy (cerebral ALD) at an early age. The Dutch Health Council recommended to screen only male newborns for ALD without identifying untreatable conditions associated with elevated C26:0-LPC. The results of the SCAN study show the feasibility of a boys-only screening algorithm that identifies males with ALD without identifying untreatable conditions.
JOURNAL OF INHERITED METABOLIC DISEASE
(2023)
Article
Cardiac & Cardiovascular Systems
Johanneke Hartog, Sandra Dijkstra, Willem Dieperink, Trynke Hoekstra, Joke Fleer, Lucas H. V. van der Woude, Pim van der Harst, Maarten Nijsten, Massimo A. Mariani, Fredrike Blokzijl
Summary: This study aimed to identify different subgroups with varying muscle strength trajectories in patients undergoing coronary artery bypass grafting (CABG) and assess their prognostic value on postoperative health-related quality of life (HR-QoL).
BMC CARDIOVASCULAR DISORDERS
(2023)
Article
Medical Laboratory Technology
Frederic M. Vaz, Youssra Jamal, Rob Barto, Michael H. Gelb, Andrea E. DeBarber, Ron A. Wevers, Marcel R. Nelen, Aad Verrips, Albert H. Bootsma, Marelle J. Bouva, Nick Kleise, Walter van der Zee, Tao He, Gajja S. Salomons, Hidde H. Huidekoper
Summary: This study aims to test the feasibility of newborn screening for Cerebrotendinous Xanthomatosis (CTX). It was found that using FIA-MS/MS or UPLC-MS/MS to measure the metabolite ratios of three CTX biomarkers is feasible for newborn screening.
CLINICA CHIMICA ACTA
(2023)
Article
Biophysics
Matteo Lancini, Paolo Spada, Ridi Muhametaj, Rick de Klerk, Lucas H. V. van der Woude, Riemer J. K. Vegter
Summary: Wheelchair ergometers are widely used and it is important to ensure their accuracy. This study presents a low-cost portable system for the dynamic metrological verification of wheelchair roller ergometers, using an instrumented reference wheel.
JOURNAL OF BIOMECHANICAL ENGINEERING-TRANSACTIONS OF THE ASME
(2023)
Article
Endocrinology & Metabolism
Sander M. M. Houten, Tetyana Dodatko, William Dwyer, Sara Violante, Hongjie Chen, Brandon Stauffer, Robert J. J. DeVita, Frederic M. Vaz, Justin R. R. Cross, Chunli Yu, Joao Leandro
Summary: This study suggests that inhibition of SBCAD enzyme can reduce the accumulation of toxic metabolic intermediates in disorders of valine and isoleucine metabolism, providing a new approach for treating related diseases.
JOURNAL OF INHERITED METABOLIC DISEASE
(2023)
Letter
Transplantation
Elisabeth L. Metry, Sander F. Garrelfs, Hessel Peters-Sengers, Frederic M. Vaz, Joost A. Bijlsma, Aegida Neradova, Michiel J. S. Oosterveld, Jaap W. Groothoff
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2023)
Article
Multidisciplinary Sciences
Ulric S. Abonie, Marloes Oldenburg, Lucas van der Woude, Florentina J. Hettinga
Summary: This study aimed to examine the differentiation in ratings of perceived exertion (RPE) between upper body and lower body exercises and their changes after upper body training. The results showed that overall RPE was higher during upper body exercises compared to lower body exercises, and both local and overall RPE increased after upper body training. During the exercises, local RPE was consistently higher than overall RPE.
Article
Sport Sciences
Rowie J. F. Janssen, Sonja de Groot, Lucas H. V. Van der Woude, Han Houdijk, Vicky L. Goosey-Tolfrey, Riemer J. K. Vegter
Summary: This study examined the impact of increased rolling resistance on wheelchair sprint performance and force-velocity characteristics. Thirteen wheelchair rugby athletes completed five sprints against escalating resistance levels. The results showed that increased resistance led to slower velocities, higher propulsion forces, and increased power outputs. Each athlete exhibited individual force-velocity profiles, suggesting potential for personalized training recommendations.
SCANDINAVIAN JOURNAL OF MEDICINE & SCIENCE IN SPORTS
(2023)
Article
Psychology, Clinical
Pim Brandenbarg, Leonie A. Krops, Bregje L. Seves, Trynke Hoekstra, Florentina J. Hettinga, Jos W. R. Twisk, Lucas H. V. van der Woude, Rienk Dekker, Femke Hoekstra
Summary: This study aimed to explore the associations between psychosocial factors and physical activity behavior in people with physical disabilities and/or chronic diseases, both between and within persons over time, and examine whether these associations differ for people initiating and maintaining physical activity behavior. The findings indicate the importance of intrinsic motivation, identified regulation, and self-efficacy in initiating and maintaining physical activity behavior.
REHABILITATION PSYCHOLOGY
(2023)
Article
Health Care Sciences & Services
Sjoukje Bouma, Martin Stevens, Lucas van der Woude, Ron Diercks, Inge van den Akker-scheek
Summary: This study aimed to identify barriers and facilitators perceived by primary and secondary healthcare professionals in implementing lifestyle-related treatment modalities in patients with hip/knee osteoarthritis. The findings suggest that focusing on societal factors rather than individual factors could improve the implementation of these treatment modalities.
Article
Engineering, Biomedical
Rafael E. A. Muchaxo, Ingrid Kouwijzer, Lucas H. V. van der Woude, Thomas W. J. Janssen, Carla F. J. Nooijen, Sonja de Groot
Summary: This study investigated the impact of performing closed kinetic chains with the lower limbs on the upper-limb pull and push strength of handcyclists. The findings suggest that athletes with milder impairments can improve their pulling performance by engaging in lower-limb closed-chain exercises.
SPORTS BIOMECHANICS
(2023)
Article
Medicine, General & Internal
Lihui Guo, Brent Appelman, Kirsten Mooij-Kalverda, Riekelt H. Houtkooper, Michel van Weeghel, Frederic M. Vaz, Annemiek Dijkhuis, Tamara Dekker, Barbara S. Smids, Jan Willem Duitman, Marianna Bugiani, Paul Brinkman, Jonne J. Sikkens, H. A. Ayesha Lavell, Rob C. I. Wuest, Michele van Vugt, Rene Lutter
Summary: This study found that the tryptophan-catabolizing enzyme indoleamine 2,3-dioxygenase-2 (IDO2) is expressed and active in patients with post-acute sequela of SARS-CoV-2 infection (PASC), and may correlate with pathophysiology. The expression of IDO2 can be triggered by SARS-CoV-2 infection and can be halted by an aryl hydrocarbon receptor (AHR) antagonist. The specific kynurenine metabolites generated by IDO2 may be related to the symptoms and pathology induced by SARS-CoV-2.
Review
Rehabilitation
Pim Brandenbarg, Femke Hoekstra, Ioulia Barakou, Bregje L. Seves, Florentina J. Hettinga, Trynke Hoekstra, Lucas H. van der Woude, Rienk Dekker, Leonie A. Krops
Summary: This scoping review examined the measurement properties of device-based instruments assessing physical activity in ambulant adults with physical disabilities and/or chronic diseases. The review found a large variability in research, highlighting the need for standardization and consensus in this field. Directions for future research were provided based on the findings.
BMC SPORTS SCIENCE MEDICINE AND REHABILITATION
(2023)
Article
Rheumatology
Sjoukje Bouma, Inge van den Akker-scheek, Dieuwke Schiphof, Lucas van der Woude, Ron Diercks, Martin Stevens
Summary: This study aims to identify and address barriers to implementing lifestyle-related treatment modalities (LRTMs) in the conservative treatment of patients with hip and/or knee osteoarthritis from the perspective of healthcare professionals (HCPs). Fourteen implementation strategies were selected, including "build a coalition" and "develop stakeholder interrelationships".
MUSCULOSKELETAL CARE
(2023)
Article
Clinical Neurology
Arthur W. D. Edridge, Gasim Abd-Elfarag, Martin Deijs, Melissa H. Broeks, Cosimo Cristella, Brandon Sie, Frederic M. Vaz, Judith J. M. Jans, Job Calis, Hans Verhoef, Ayse Demir, Sven Poppert, Beatrice Nickel, Alje van Dam, Boy Sebit, Maarten J. Titulaer, Jaco J. Verweij, Menno D. de Jong, Tom van Gool, Brian Faragher, Nanda M. Verhoeven-Duif, Stephen J. Elledge, Lia van der Hoek, Michael Boele van Hensbroek
Summary: Nodding syndrome is a neglected, disabling and potentially fatal epileptic disorder that primarily affects individuals in Eastern sub-Saharan Africa. Our study suggests that Mansonella perstans infection, Necator americanus infection, higher antimalarial seroreactivity, higher vitamin E concentration, and lower vitamin B12 concentration are associated with an increased risk of nodding syndrome.
BRAIN COMMUNICATIONS
(2023)