Article
Biochemistry & Molecular Biology
David M. Kosek, Elnaz Banijamali, Walter Becker, Katja Petzold, Emma R. Andersson
Summary: This study investigates the combined effect of 3'-pairing and secondary structures in mRNAs on miRNA repression efficiency. The results show that 3'-pairing can compensate for low seed-binding site accessibility and enable repression of otherwise ineffective sites. The study also reveals that miRNA 3'-pairing regions can base-pair with nucleotides far upstream of the seed-binding site and tolerate both hairpins and unstructured bulges within the target site.
NUCLEIC ACIDS RESEARCH
(2023)
Article
Chemistry, Multidisciplinary
Batoul Srour, Sylvain Gervason, Maren Hellen Hoock, Beata Monfort, Kristian Want, Djabir Larkem, Nadine Trabelsi, Gautier Landrot, Andrea Zitolo, Emiliano Fonda, Emilien Etienne, Guillaume Gerbaud, Christina Sophia Mueller, Jonathan Oltmanns, Jesse B. Gordon, Vishal Yadav, Malgorzata Kleczewska, Marcin Jelen, Michel B. Toledano, Rafal Dutkiewicz, David P. Goldberg, Volker Schuenemann, Bruno Guigliarelli, Benedicte Burlat, Christina Sizun, Benoit D'Autreaux
Summary: The study demonstrates that iron binds to the assembly site of ISCU proteins via one or two cysteines, forming 1-Cys and 2-Cys forms, respectively. This provides a structural basis to elucidate the Fe-S cluster assembly process.
JOURNAL OF THE AMERICAN CHEMICAL SOCIETY
(2022)
Review
Biochemistry & Molecular Biology
Courtney J. Campbell, Ashley E. Pall, Akshata R. Naik, Lindsey N. Thompson, Timothy L. Stemmler
Summary: Iron-sulfur clusters are crucial for cellular activities and the ISC pathway serves as the central mechanism for providing 2Fe-2S clusters in eukaryotes. Study of the NIAUF complex reveals insights into the coordinated mechanism of Fe-S cluster assembly.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Spectroscopy
Mingxi Fang, Xiaoyu Zhou, Shaocai Wang, Yinshuang Yang, Yueting Cheng, Boling Wang, Xiaoqian Rong, Xiuli Zhang, Kai Xu, Yibin Zhang, Shaohui Zheng
Summary: A novel near infrared fluorescent probe (FNIR-pH) was synthesized for measurement of mitochondrial pH, allowing real-time monitoring of changes in cellular mitochondrial pH and sensitive detection of mitophagy. The probe exhibited excellent selectivity, photostability, and low cytotoxicity, making it suitable for in vivo imaging.
SPECTROCHIMICA ACTA PART A-MOLECULAR AND BIOMOLECULAR SPECTROSCOPY
(2023)
Article
Endocrinology & Metabolism
Marit Schwantje, Merel S. Ebberink, Mirjam Doolaard, Jos P. N. Ruiter, Sabine A. Fuchs, Niklas Darin, Carola Hedberg-Oldfors, Luc Regal, Laura Donker Kaat, Hidde H. Huidekoper, Simon Olpin, Duncan Cole, Stuart J. Moat, Gepke Visser, Sacha Ferdinandusse
Summary: This study describes five patients with thermo-sensitive MTP deficiency, with onset in childhood and myopathic symptoms triggered by fever or exercise. The absence of characteristic acylcarnitine abnormalities poses a risk for delayed diagnosis, while preventive measures can improve symptoms.
JOURNAL OF INHERITED METABOLIC DISEASE
(2022)
Review
Biochemistry & Molecular Biology
Nunziata Maio, Anshika Jain, Tracey A. Rouault
CURRENT OPINION IN CHEMICAL BIOLOGY
(2020)
Editorial Material
Biochemistry & Molecular Biology
Tracey A. Rouault, Nunziata Maio
Article
Biochemistry & Molecular Biology
Anshika Jain, Anamika Singh, Nunziata Maio, Tracey A. Rouault
HUMAN MOLECULAR GENETICS
(2020)
Article
Hematology
Manik C. Ghosh, De-Liang Zhang, Wade H. Ollivierre, Audrey Noguchi, Danielle A. Springer, W. Marston Linehan, Tracey A. Rouault
Summary: The study demonstrates that treatment with the HIF-2 alpha inhibitor MK-6482 is effective in reversing polycythemia and mitigating pulmonary hypertension, cardiac fibrosis, and pulmonary fibrosis by reducing the expression of HIF-2 alpha and its target genes.
Article
Biochemistry & Molecular Biology
Daniel R. Crooks, Nunziata Maio, Martin Lang, Christopher J. Ricketts, Cathy D. Vocke, Sandeep Gurram, Sevilay Turan, Yun-Young Kim, G. Mariah Cawthon, Ferri Sohelian, Natalia De Val, Ruth M. Pfeiffer, Parthav Jailwala, Mayank Tandon, Bao Tran, Teresa W-M Fan, Andrew N. Lane, Thomas Ried, Darawalee Wangsa, Ashkan A. Malayeri, Maria J. Merino, Youfeng Yang, Jordan L. Meier, Mark W. Ball, Tracey A. Rouault, Ramaprasad Srinivasan, W. Marston Linehan
Summary: Understanding the mechanism of Warburg shift to aerobic glycolysis is essential for defining the metabolic basis of cancer. HLRCC, an aggressive cancer, is characterized by impaired mitochondrial respiratory chain, loss of expression of mtDNA-encoded subunits, and accumulation of fumarate, leading to the promotion of aerobic glycolysis and disease progression. Accumulation of fumarate in HLRCC tumors disrupts mtDNA replication and proofreading, causing loss of respiratory chain components.
Article
Multidisciplinary Sciences
Nunziata Maio, Bernard A. P. Lafont, Debangsu Sil, Yan Li, J. Martin Bollinger, Carsten Krebs, Theodore C. Pierson, W. Marston Linehan, Tracey A. Rouault
Summary: Research has shown that the catalytic subunit nsp12 of the SARS-CoV-2 RdRp complex ligates two iron-sulfur metal cofactors which are essential for viral replication and interaction with the viral helicase. Oxidation of these clusters by TEMPOL inhibits RdRp activity and blocks SARS-CoV-2 replication in cell culture, indicating that these iron-sulfur clusters could be targeted for therapy of COVID-19.
Article
Hematology
Nunziata Maio, De-Liang Zhang, Manik C. Ghosh, Anshika Jain, Anna M. SantaMaria, Tracey A. Rouault
Summary: Humans and other vertebrates have evolved effective mechanisms to maintain iron balance through the regulation of iron concentration and utilization at the cellular and systemic levels, involving iron-regulatory hormone hepcidin and iron regulatory proteins.
SEMINARS IN HEMATOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Nunziata Maio, Tracey A. Rouault
Article
Biochemistry & Molecular Biology
Nunziata Maio, Tracey A. Rouault
Article
Multidisciplinary Sciences
Forson Chan, Simon Lai, Marcus Pieterman, Lisa Richardson, Amanda Singh, Jocelynn Peters, Alex Toy, Caroline Piccininni, Taiysa Rouault, Kristie Wong, James K. Quong, Adrienne T. Wakabayashi, Anna Pawelec-Brzychczy
Summary: A new prototype symptom checker was developed and tested for safety and accuracy in real patient scenarios. The study found that the prototype outperformed patients in deciding the most appropriate treatment setting for medical issues. Compliance with the symptom checker recommendations could significantly reduce unnecessary hospital visits while maintaining accuracy and safety outcomes.
Article
Multidisciplinary Sciences
Jin-Seon Yook, Mikyoung You, Jiyoung Kim, Ashley M. Toney, Rong Fan, Bhanwar Lal Puniya, Tomas Helikar, Sophie Vaulont, Jean-Christophe Deschemin, Meshail Okla, Liwei Xie, Manik C. Ghosh, Tracey A. Rouault, Jaekwon Lee, Soonkyu Chung
Summary: This study investigates the iron-regulatory network underlying beige adipogenesis, showing that thermogenic stimulus leads to redistribution of iron into beige fat through interplay between adipocyte iron demands and systemic iron liberation. Disruption of systemic iron homeostasis impairs beige fat development, suggesting coordinated interplay between renal hypoxia and hepcidin down-regulation as a key mechanism for activating adaptive thermogenesis.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Review
Biochemistry & Molecular Biology
Christina A. Porras, Tracey A. Rouault
Summary: This review provides an overview of the role of iron homeostasis disruption in various neurological disorders, discussing the consequences of both iron accumulation and deficiency. It also examines the history of animal models and presents a comparison of patients with a newly discovered neurodegenerative disorder caused by mutations in iron regulatory protein 2.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Nunziata Maio, Sara Cherry, David C. Schultz, Brett L. Hurst, W. Marston Linehan, Tracey A. Rouault
Summary: TEMPOL has been shown to inhibit the RNA-dependent RNA polymerase of SARS-CoV-2 and effectively block viral replication in the Syrian hamster model. These findings support the potential application of TEMPOL as a highly efficacious antiviral against COVID-19 in humans.
Article
Multidisciplinary Sciences
Wing-Hang Tong, Hayden Ollivierre, Audrey Noguchi, Manik C. Ghosh, Danielle A. Springer, Tracey A. Rouault
Summary: Cardiomyopathy is a primary cause of death in Friedreich ataxia patients with defective iron-sulfur cluster biogenesis. The mTOR and AKT signaling cascades play crucial roles in regulating cardiovascular growth and homeostasis, with mTOR and AKT hyperactivations possibly as part of the metabolic stress response to ISC deficiencies.
Article
Clinical Neurology
Nunziata Maio, Russell P. Saneto, Richard Steet, Marcio A. Sotero de Menezes, Cindy Skinner, Tracey A. Rouault
Summary: Altered brain iron homeostasis can contribute to neurodegeneration. Mutations in the IREB2 gene can cause a newly characterized type of early onset progressive neurodegenerative disease, impacting iron metabolism regulation.
BRAIN COMMUNICATIONS
(2022)