Article
Biochemistry & Molecular Biology
Maria Petrosino, Karim Zuhra, Jola Kopec, Andrew Hutchin, Csaba Szabo, Tomas Majtan
Summary: In this study, the binding and inhibitory mechanism of the CBS inhibitor AOAA were characterized. It was found that AOAA can be replaced by serine, which allows for the continuation of the catalytic cycle. Serine rescued the activity and function of AOAA-inhibited CBS. The results demonstrate the complexity of using AOAA as a CBS inhibitor and highlight the need for a potent, selective, and specific pharmacological CBS inhibitor.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Zhitao Mao, Xin Zhao, Xue Yang, Peiji Zhang, Jiawei Du, Qianqian Yuan, Hongwu Ma
Summary: Genome-scale metabolic models (GEMs) are widely used for predicting the phenotypes of microorganisms. However, the lack of other constraints in the stoichiometric model often limits the accessibility of the metabolic solution space. In this study, we developed an enzyme-constrained metabolic network model (ECMpy) and applied it to Escherichia coli (E. coli) to improve the accuracy of phenotype predictions. By considering the total enzyme amount constraint, protein subunit composition, and enzyme kinetic parameters, we were able to better predict the overflow metabolism and growth rates of E. coli. The enzyme-constrained model revealed the tradeoff between enzyme usage efficiency and biomass yield, providing valuable insights for metabolic engineering.
Article
Agricultural Engineering
Misa Mojca Cajnko, Jost Oblak, Miha Grilc, Blaz Likozar
Summary: Enzymes play a crucial role in lignin valorization, with different enzymes involved in various elementary reaction mechanisms. Degradation of lignin depends on enzyme activity and the interaction with proteins in ways that target specific covalent bonds in lignin. Various factors influence enzymatic activity, which must be taken into consideration for optimal functionality and synthesis yields.
BIORESOURCE TECHNOLOGY
(2021)
Article
Agriculture, Multidisciplinary
Zheng Zhang, Jia Wei, Man Wang, Jian Zhang, Bin Wu
Summary: The study showed that SO2 treatment helps maintain the postharvest quality of table grapes by increasing sulfur compound content, enzyme activity, and modulating gene expression related to sulfur metabolism.
JOURNAL OF THE SCIENCE OF FOOD AND AGRICULTURE
(2022)
Article
Chemistry, Multidisciplinary
Yaojie Lei, Can Wu, Xinxin Lu, Weibo Hua, Shaobo Li, Yaru Liang, Hanwen Liu, Wei-Hong Lai, Qinfeng Gu, Xiaolan Cai, Nana Wang, Yun-Xiao Wang, Shu-Lei Chou, Hua-Kun Liu, Guoxiu Wang, Shi-Xue Dou
Summary: It is crucial to regulate S activity dynamically for efficient and stable room-temperature sodium-sulfur (RT/Na-S) batteries. In this study, cobalt sulfide is used as an electron reservoir to enhance the activity of sulfur cathodes, and cobalt single atoms serve as double-end binding sites for stable S conversion. The rational construction of CoS2 electron reservoir enables the direct reduction of S to short-chain sodium polysulfides (Na2S4) through a streamlined redox path. Cobalt single atoms synergistically work with the electron reservoir to reinforce the streamlined redox path, immobilize in situ formed long-chain products, and catalyze their conversion, leading to high S utilization and sustainable cycling stability. The developed sulfur cathodes exhibit superior rate performance and high cycling capacity retention.
ANGEWANDTE CHEMIE-INTERNATIONAL EDITION
(2022)
Article
Pharmacology & Pharmacy
Marco Albrecht, Yuri Kogan, Dagmar Kulms, Thomas Sauter
Summary: Dabrafenib inhibits cell proliferation in metastatic melanoma, but its effectiveness is limited due to drug resistance and relapse. Using a pharmacokinetic model, we predict the drug concentrations after long-term accumulation and found that it has limited effect on melanoma cells.
Article
Chemistry, Multidisciplinary
Yang Chao, Akash Krishna, Muthusamy Subramaniam, Dong-Dong Liang, Sidharam P. Pujari, Andrew C-H Sue, Guanna Li, Fedor M. Miloserdov, Han Zuilhof
Summary: The SuFEx reaction between sulfonimidoyl fluorides and phenols, sulfonimidates, exhibits dynamic covalent chemistry with other phenols. This enantiospecific reaction can be completed at room temperature within minutes and with high yields, making it useful for asymmetric synthesis and sustainable polymer production. Its broad scope further extends the applications of SuFEx and related click chemistries.
ANGEWANDTE CHEMIE-INTERNATIONAL EDITION
(2022)
Article
Cell Biology
Paulina Sosicka, Bobby G. Ng, Lauren E. Pepi, Asif Shajahan, Maurice Wong, David A. Scott, Kenjiroo Matsumoto, Zhi-Jie Xia, Carlito B. Lebrilla, Robert S. Haltiwanger, Parastoo Azadi, Hudson H. Freeze
Summary: Cells selectively use activated GDP-fucose from multiple, separate pools for glycosylation, dispelling the concept of a single, homogenous precursor pool. This discovery provides novel perspectives on monosaccharide metabolism.
JOURNAL OF CELL BIOLOGY
(2022)
Article
Chemistry, Multidisciplinary
Azimberdy Besya, Shodhan Rao
Summary: The focus of this study is on the approximation method and validity conditions of tQSSA for multiple substrate reactions. By introducing a linearization method, an analytical solution for tQSSA equations of multiple substrate reactions is obtained, and the validity conditions of tQSSA are determined using the singular perturbation analysis method.
JOURNAL OF MATHEMATICAL CHEMISTRY
(2022)
Editorial Material
Biology
Mingyu Shin, Jiwon Shim
Summary: A gene involved in hypoxia response has been found to protect insect muscles during migratory flight in a non-oxygen dependent manner.
Article
Chemistry, Multidisciplinary
Claudio M. Nunes, Jose P. L. Roque, Srinivas Doddipatla, Samuel A. Wood, Robert J. McMahon, Rui Fausto
Summary: This study presents a new example of chemical reactivity governed by quantum tunneling and highlights the limitations of classical theories. The different conformers of a 2-formylphenylnitrene underwent spontaneous rearrangement to different products, and the kinetics measurements provided evidence of concomitant tunneling reactions.
JOURNAL OF THE AMERICAN CHEMICAL SOCIETY
(2022)
Review
Nutrition & Dietetics
Chelsea Truitt, Wouter D. Hoff, Ratnakar Deole
Summary: Homocystinuria is a medical condition caused by a deficiency in enzymes, leading to elevated levels of homocysteine. Betaine is a treatment option that can help lower homocysteine levels to within normal ranges.
FRONTIERS IN NUTRITION
(2021)
Article
Chemistry, Organic
Shuangrong Liu, Zhimin Ying, Qi Zhu, Xiuwen Zhong, Jie Cen, You Wu, Yongping Yu, Jiaan Shao, Wenteng Chen
Summary: This study reveals a step-economical nitrite-triggered cycloaddition reaction for the modular assembly of isoxazoles (or isoxazolines) from bromide and alkyne/alkene. The reaction offers high regio-selectivity, excellent efficiency, wide functionality tolerance, biocompatible conditions, and operational simplicity, making it suitable for the direct diversification of pharmaceuticals and bio-relevant motifs.
ORGANIC CHEMISTRY FRONTIERS
(2023)
Article
Agricultural Engineering
Kaiquan Wang, Mahmood Qaisar, Bilong Chen, Jinghong Xiao, Jing Cai
Summary: The study found that adjusting the hydraulic retention time appropriately can increase the production of sulfide (S-0) and improve the simultaneous removal of sulfide and nitrite. Under different HRT conditions, bacterial community richness and key gene expression will change, affecting the performance of the recirculation system.
BIORESOURCE TECHNOLOGY
(2022)
Article
Chemistry, Physical
Yingze Song, Hua Gao, Menglei Wang, Le Chen, Xuan Cao, Lixian Song, Xiaohong Liu, Wenlong Cai, Jingyu Sun, Wei Zhang
Summary: In this study, the intrinsic defect of graphene was modified using plasma irradiation, which effectively improved the performance of lithium-sulfur batteries, leading to enhanced cyclability and areal capacity.
Article
Biochemistry & Molecular Biology
Theodora Panagaki, Laura Lozano-Montes, Lucia Janickova, Karim Zuhra, Marcell P. Szabo, Tomas Majtan, Gregor Rainer, Damien Marechal, Yann Herault, Csaba Szabo
Summary: This study investigates the functional role of the cystathionine-beta-synthase (CBS)/hydrogen sulfide (H2S) pathway in the pathogenesis of brain wave pattern alterations and neurobehavioral dysfunction in a rat model of Down syndrome (DS). The overexpression of CBS and subsequent overproduction of H2S in the brain of DS rats were observed. The study suggests that the CBS/H2S pathway contributes to the neurological dysfunction in DS through dysregulation of cellular bioenergetics and gene expression.
Article
Endocrinology & Metabolism
Ulrike Muetze, Florian Gleich, Sven F. Garbade, Celine Plisson, Luis Aldamiz-Echevarria, Francisco Arrieta, Diana Ballhausen, Matthias Zielonka, Danijela Petkovic Ramadza, Matthias R. Baumgartner, Aline Cano, Maria Concepcion Garcia Jimenez, Carlo Dionisi-Vici, Pavel Jesina, Henk J. Blom, Maria Luz Couce, Silvia Meavilla Olivas, Karine Mention, Fanny Mochel, Andrew A. M. Morris, Helen Mundy, Isabelle Redonnet-Vernhet, Saikat Santra, Manuel Schiff, Aude Servais, Isidro Vitoria, Martina Huemer, Viktor Kozich, Stefan Koelker
Summary: This study evaluates the postauthorization safety study (PASS) for betaine anhydrous, conducted as a public-private partnership (PPP) between a network and registry for specific diseases and the marketing authorization holder. The study found that although some individuals exceeded the recommended dose, no new or potential risks were identified. The study also resulted in revised recommendations for betaine anhydrous dosage.
JOURNAL OF INHERITED METABOLIC DISEASE
(2022)
Article
Endocrinology & Metabolism
Mathilde Yverneau, Stephanie Leroux, Apolline Imbard, Florian Gleich, Alina Arion, Caroline Moreau, Marie-Cecile Nassogne, Marie Szymanowski, Marine Tardieu, Guy Touati, Maria Bueno, Kimberly A. Chapman, Yin-Hsiu Chien, Martina Huemer, Pavel Jesina, Mirian C. H. Janssen, Stefan Koelker, Viktor Kozich, Christian Lavigne, Allan Meldgaard Lund, Fanny Mochel, Andrew Morris, Monica Ruiz Pons, Gloria Liliana Porras-Hurtado, Jean-Francois Benoist, Lena Damaj, Manuel Schiff
Summary: This study describes the clinical and laboratory parameters of early-onset MTHFR deficiency and identifies predictive factors for severe neurodevelopmental outcomes. The findings suggest that early diagnosis and appropriate treatment have benefits in improving outcomes.
JOURNAL OF INHERITED METABOLIC DISEASE
(2022)
Article
Genetics & Heredity
Aixa Gonzalez, Geoffrey Hughes Smith, Michael J. Gambello, Jitka Sokolova, Viktor Kozich, Hong Li
Summary: This study aims to identify undiagnosed inborn errors of metabolism (IEM) in adults with elevated homocysteine levels, and offer specific treatment to improve outcomes. Through retrospective analysis, one patient with homocystinuria was diagnosed and had improved health with lowered homocysteine levels through treatment.
AMERICAN JOURNAL OF MEDICAL GENETICS PART A
(2023)
Article
Cell Biology
Sailendra N. Nichenametla, Dwight A. L. Mattocks, Diana Cooke, Vishal Midya, Virginia L. Malloy, Wilfredo Mansilla, Bente Ovrebo, Cheryl Turner, Nasser E. Bastani, Jitka Sokolova, Marketa Pavlikova, John P. Richie, Anna K. Shoveller, Helga Refsum, Thomas Olsen, Kathrine J. Vinknes, Viktor Kozich, Gene P. Ables
Summary: Decreasing the intake of methionine has significant anti-adiposity effects in rodents but only modest effects in humans. The study found that the anti-adiposity effects of dietary restriction were due to cysteine restriction, which increased serinogenesis and reduced fatty acid reesterification and triglyceride synthesis. This finding may have implications for the treatment of hypertriglyceridemia.
Review
Pharmacology & Pharmacy
Tomas Majtan, Viktor Kozich, Warren D. Kruger
Summary: Homocystinuria, a common metabolic disorder, is characterized by increased homocysteine levels, leading to various clinical complications. Current treatment primarily involves dietary interventions, but alternative therapies such as enzyme and gene therapies have been explored.
BRITISH JOURNAL OF PHARMACOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Viktor Kozich, Bernd C. Schwahn, Jitka Sokolova, Michaela Krizkova, Tamas Ditroi, Jakub Krijt, Youssef Khalil, Tomas Krizek, Tereza Vaculikova-Fantlova, Blanka Stiburkova, Philippa Mills, Peter Clayton, Kristyna Barvikova, Holger Blessing, Jolanta Sykut-Cegielska, Carlo Dionisi-Vici, Serena Gasperini, Angeles Garcia-Cazorla, Tobias B. Haack, Tomas Honzik, Pavel Jesina, Alice Kuster, Lucia Laugwitz, Diego Martinelli, Francesco Porta, Rene Santer, Guenter Schwarz, Peter Nagy
Summary: Regulation of H2S homeostasis in humans is not well understood. By studying patients with genetic defects, the importance of certain enzymes in H2S synthesis and catabolism was assessed. The findings suggest the presence of control and compensatory mechanisms in the maintenance of H2S homeostasis.
Article
Nutrition & Dietetics
Elena C. Tore, Simone J. P. M. Eussen, Nasser E. Bastani, Pieter C. Dagnelie, Amany K. Elshorbagy, Pol Grootswagers, Viktor Kozich, Thomas Olsen, Helga Refsum, Kjetil Retterstol, Coen D. A. Stehouwer, Emma T. K. Stolt, Kathrine J. Vinknes, Marleen M. J. van Greevenbroek
Summary: The study aimed to investigate the influence of dietary intake and nutrients on plasma amino acid concentrations. The results showed that intake of total amino acids and total proteins was positively associated with plasma tCys and cystathionine, with stronger associations in women and those with normal body weight. Higher intake of cysteine and plant proteins was associated with lower plasma tHcy and higher cystathionine. Higher methionine intake was associated with lower plasma tGSH, whereas cysteine intake was positively associated with tGSH. Higher intake of methionine and animal proteins was associated with higher plasma taurine. Better diet quality was consistently related to lower plasma tHcy concentrations, but it was not associated with the other SAAs.
JOURNAL OF NUTRITION
(2023)
Review
Genetics & Heredity
Matej Mlinaric, James R. Bonham, Viktor Kozich, Stefan Koelker, Ondrej Majek, Tadej Battelino, Ana Drole Torkar, Vanesa Koracin, Dasa Perko, Ziga Iztok Remec, Barbka Repic Lampret, Maurizio Scarpa, Peter C. J. I. Schielen, Rolf H. Zetterstrom, Urh Groselj
Summary: The COVID-19 pandemic had negative impacts on the newborn screening programs, causing missed cases of inherited metabolic diseases due to decreased diagnostic quality. Contingency plans were implemented to address the problems emerged at the start of the pandemic, including staff shortage and modified work schedules. Adaptations and modifications were made in sample collection, resource sharing, and telemedicine utilization to ensure the continuity of the screening programs.
INTERNATIONAL JOURNAL OF NEONATAL SCREENING
(2023)
Article
Biochemistry & Molecular Biology
Renata Collard, Tomas Majtan
Summary: Homocystinuria is an inherited metabolic disorder caused by lack of cystathionine beta-synthase (CBS) activity. This study found that pathogenic CBS variants result in dysregulation of gene expression of small heat shock proteins and HSP40 family members, as well as endoplasmic reticulum stress and protein degradation. Manipulation of cellular proteostasis could rescue CBS variant function and potentially serve as a therapeutic approach for treating HCU.
MOLECULAR AND CELLULAR BIOLOGY
(2023)
Review
Genetics & Heredity
Jaka Sikonja, Urh Groselj, Maurizio Scarpa, Giancarlo la Marca, David Cheillan, Stefan Koelker, Rolf H. Zetterstrom, Viktor Kozich, Yann Le Cam, Gulcin Gumus, Valentina Bottarelli, Mirjam van der Burg, Eugenie Dekkers, Tadej Battelino, Johan Prevot, Peter C. J. Schielen, James R. Bonham
Summary: Although individual rare disorders are uncommon, together they affect more than 30 million people in Europe, causing a substantial burden on public health. Newborn screening (NBS) can detect rare conditions in asymptomatic children, leading to early treatment and improved long-term outcomes. However, the availability and conduct of NBS programmes vary across Europe, highlighting the need for improvement to ensure equity and high-quality screening.
INTERNATIONAL JOURNAL OF NEONATAL SCREENING
(2022)