Article
Multidisciplinary Sciences
Abigael Muchenditsi, C. Conover Talbot, Aline Gottlieb, Haojun Yang, Byunghak Kang, Tatiana Boronina, Robert Cole, Li Wang, Som Dev, James P. Hamilton, Svetlana Lutsenko
Summary: The study found that global inactivation of Atp7b enhances and specifically modifies the hepatocyte response to copper overload, while the loss of Atp7b only in hepatocytes causes dysregulation of lipid and nucleic acid metabolism.
SCIENTIFIC REPORTS
(2021)
Article
Biochemistry & Molecular Biology
Marko Rodewald, J. Mikko Rautiainen, Helmar Goerls, Raija Oilunkaniemi, Wolfgang Weigand, Risto S. Laitinen
Summary: A new platinum complex was synthesized and its crystal structure was analyzed. It was found that the ligand undergoes isomerization and forms a dynamic equilibrium during the dissolution process. Two other platinum complexes were also successfully synthesized and their structures were characterized. The bonding and formation of these complexes were further discussed through theoretical calculations.
Article
Medicine, Research & Experimental
Wai-In Ho, Yang Hu, Chi-Wa Cheng, Rui Wei, Jiayin Yang, Na Li, Ka-Wing Au, Yiu-Lam Tse, Qiuyun Wang, Kwong-Man Ng, Miguel A. Esteban, Hung-Fat Tse
Summary: In this study, liposome-encapsulated curcumin (LEC) was used to treat a mouse model of Wilson's disease (WD). The treatment was found to attenuate liver injuries, restore lipid metabolism, decrease inflammation and fibrosis, and reduce hepatosplenomegaly in the mice. The mechanism of action was through the suppression of HMGB1-mediated hepatic and systemic inflammation.
BIOMEDICINE & PHARMACOTHERAPY
(2022)
Article
Biochemistry & Molecular Biology
Hiroaki Ishida, Hans J. Vogel, Alex C. Conner, Philip Kitchen, Roslyn M. Bill, Justin A. MacDonald
Summary: This study assessed the CaM-binding properties of AQP4, finding two putative CaM-binding domains in the cytoplasmic regions. The N-terminal CBD of AQP4 interacted predominantly with the N-lobe of CaM, while the C-terminal AQP4 peptide interacted with both the C-and N-lobes of CaM. The data support a unique adapter protein binding mode for CaM with AQP4.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES
(2022)
Article
Gastroenterology & Hepatology
Saleh A. Alqahtani, Rose Chami, Dua Abuquteish, Shannon M. Vandriel, Charesse Yap, Liyana Kukkadi, Aishwarya Parmar, Amrita Mundh, Eve A. Roberts, Binita M. Kamath, Iram Siddiqui
Summary: This study compared the histological features of Wilson disease (WD) in children with non-alcoholic fatty liver disease (NAFLD) and autoimmune hepatitis (AIH). It found that there was considerable overlap in the light microscopic findings, but ultrastructural findings of mitochondrial abnormalities were important in distinguishing WD from NAFLD and AIH.
LIVER INTERNATIONAL
(2022)
Review
Cell Biology
Som Dev, Robert L. Kruse, James P. Hamilton, Svetlana Lutsenko
Summary: This review summarizes recent advances in the characterization of Wilson disease pathophysiology and discusses emerging targets for improving diagnosis and treatment.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Review
Immunology
Yixuan Zhou, Leiliang Zhang
Summary: This review discusses the key features and trafficking mechanisms of copper transporters ATP7A/B and examines the intricate interplay between microbes and copper metabolism. It highlights how microbes can perturb copper homeostasis through interactions with host factors.
FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY
(2023)
Article
Multidisciplinary Sciences
Som Dev, Abigael Muchenditsi, Aline Gottlieb, Pragney Deme, Sean Murphy, Kathleen L. Gabrielson, Yixuan Dong, Robert Hughes, Norman J. Haughey, James P. Hamilton, Svetlana Lutsenko
Summary: This study identified an important regulatory pathway in Wilson disease with copper metabolism abnormalities, which can improve liver damage and restore function.
Article
Chemistry, Inorganic & Nuclear
M. V. Pozharov, O. V. Fedotova, I. V. Kanevskaya, E. M. Arzyamova
Summary: The study aimed to investigate tautomeric forms of arylmethylenbis-4hydroxy-6-methyl-2H-pyran-2-ones, finding that the ligands primarily exist in the dienol tautomer form. Copper complexes with these ligands were synthesized and characterized using NMR, quantum chemical, and thermogravimetric methods.
INORGANICA CHIMICA ACTA
(2021)
Review
Biochemistry & Molecular Biology
Bryce Blades, Scott Ayton, Ya Hui Hung, Ashley Bush, Sharon La Fontaine
Summary: The complex and inverse relationship between copper and lipid metabolism has implications for diseases involving dyslipidemia. Understanding these pathways may provide druggable targets for therapeutic intervention. Tissue-specific roles of the copper regulatory protein ATP7B further elucidate the association between copper and lipid metabolism.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2021)
Review
Cell Biology
Svetlana Lutsenko
Summary: Copper homeostasis is crucial for the development and function of organisms, with misbalance leading to serious pathologies. Mammalian Cu(I) transporters play a key role in regulating intracellular copper fluxes, with various regulatory proteins contributing to the dynamic and cell-specific nature of the copper transport networks. The comparison of copper transport mechanisms in the liver and intestine highlights the distinct composition and regulatory responses of their transporters to changing copper levels.
JOURNAL OF CELL SCIENCE
(2021)
Article
Genetics & Heredity
Amelie Stalke, Annika Behrendt, Finja Hennig, Holger Gohlke, Nicole Buhl, Thea Reinkens, Ulrich Baumann, Brigitte Schlegelberger, Thomas Illig, Eva-Doreen Pfister, Britta Skawran
Summary: This study describes the clinical characteristics of six Wilson's disease patients and functionally characterizes five ATP7B missense variants detected in these patients. The results provide insights into the pathomechanism of these variants and reclassify two variants of unknown significance and three likely pathogenic variants.
Article
Chemistry, Multidisciplinary
Monika Chwastek, Piotr Cmoch, Agnieszka Szumna
Summary: Spatial sequestration of molecules is essential for the complexity of biological systems, and chemists have created artificial nanocontainers to mimic this concept and apply it in various fields. In this study, large closed-shell species were formed by interactions between polyphenolic macrocycles and anions, resulting in even larger capsular structures. This self-assembly is effective in nonpolar solvents, but requires initiation by mechanochemistry.
JOURNAL OF THE AMERICAN CHEMICAL SOCIETY
(2022)
Article
Clinical Neurology
Zi-Wei Zheng, Meng-Hui Xu, Chuan-Bin Sun, Zhi-Ying Wu, Yi Dong
Summary: This study reported a case of a patient with Wilson's disease (WD) who presented with acute-onset visual impairment. By reviewing previous cases, it was found that WD has complex clinical manifestations. Although the etiology behind visual impairment remains uncertain, the possibility of WD should be considered through neuropsychiatric and hepatic symptoms, corneal K-F rings, decreased serum ceruloplasmin, and low likelihood or exclusion of other causes. Clinicians need to recognize this rare manifestation and provide appropriate treatment to avoid misdiagnosis and overtreatment.
FRONTIERS IN NEUROLOGY
(2022)
Article
Biophysics
Andres T. Cavazos, Edward Ross Pennington, Sahil Dadoo, Kymberly M. Gowdy, Stephen R. Wassall, Saame Raza Shaikh
Summary: Long-chain polyunsaturated fatty acids are prone to oxidation and play a role in controlling inflammatory response. The mechanism of action by which oxidized PUFAs exert their functional effects remains unclear.
BIOPHYSICAL JOURNAL
(2023)
Letter
Hematology
Tzu-Fei Wang, Jennifer E. Dawson, Julie. D. Forman-Kay, Walter H. A. Kahr, Suzan Williams, Anthony K. Chan, Riten Kumar
BRITISH JOURNAL OF HAEMATOLOGY
(2017)
Article
Biochemistry & Molecular Biology
P. Andrew Chong, Julie D. Forman-Kay
CURRENT OPINION IN STRUCTURAL BIOLOGY
(2016)
Review
Biochemistry & Molecular Biology
Alaji Bah, Julie D. Forman-Kay
JOURNAL OF BIOLOGICAL CHEMISTRY
(2016)
Review
Chemistry, Multidisciplinary
Asmit Bhowmick, David H. Brookes, Shane R. Yost, H. Jane Dyson, Julie D. Forman-Kay, Daniel Gunter, Martin Head-Gordon, Gregory L. Hura, Vijay S. Pande, David E. Wemmer, Peter E. Wright, Teresa Head-Gordon
JOURNAL OF THE AMERICAN CHEMICAL SOCIETY
(2016)
Article
Medicine, Research & Experimental
Amandeep K. Gakhal, Timothy J. Jensen, Zoltan Bozoky, Ariel Roldan, Gergely L. Lukacs, Julie Forman-Kay, John R. Riordan, Sachdev S. Sidhu
Article
Biochemistry & Molecular Biology
Robert M. Vernon, P. Andrew Chong, Hong Lin, Zhengrong Yang, Qingxian Zhou, Andrei A. Aleksandrov, Jennifer E. Dawson, John R. Riordan, Christie G. Brouillette, Patrick H. Thibodeau, Julie D. Forman-Kay
JOURNAL OF BIOLOGICAL CHEMISTRY
(2017)
Article
Pharmacology & Pharmacy
Rhea P. Hudson, Jennifer E. Dawson, P. Andrew Chong, Zhengrong Yang, Linda Millen, Philip J. Thomas, Christie G. Brouillette, Julie D. Forman-Kay
MOLECULAR PHARMACOLOGY
(2017)
Editorial Material
Multidisciplinary Sciences
P. Andrew Chong, Julie D. Forman-Kay
Article
Multidisciplinary Sciences
Zoltan Bozoky, Saumel Ahmadi, Tal Milman, Tae Hun Kim, Kai Du, Michelle Di Paola, Stan Pasyk, Roman Pekhletski, Jacob P. Keller, Christine E. Bear, Julie D. Forman-Kay
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2017)
Article
Multidisciplinary Sciences
David P. Scanlon, Alaji Bah, Mickael Krzeminski, Wenbo Zhang, Heather L. Leduc-Pessah, Yi Na Dong, Julie D. Forman-Kay, Michael W. Salter
NATURE COMMUNICATIONS
(2017)
Article
Multidisciplinary Sciences
Veronika Csizmok, Stephen Orlicky, Jing Cheng, Jianhui Song, Alaji Bah, Neda Delgoshaie, Hong Lin, Tanja Mittag, Frank Sicheri, Hue Sun Chan, Mike Tyers, Julie D. Forman-Kay
NATURE COMMUNICATIONS
(2017)
Article
Genetics & Heredity
Saumel Ahmadi, Zoltan Bozoky, Michelle Di Paola, Sunny Xia, Canhui Li, Amy P. Wong, Leigh Wellhauser, Steven V. Molinski, Wan Ip, Hong Ouyang, Julie Avolio, Julie D. Forman-Kay, Felix Ratjen, Jeremy A. Hirota, Johanna Rommens, Janet Rossant, Tanja Gonska, Theo J. Moraes, Christine E. Bear
NPJ GENOMIC MEDICINE
(2017)
Article
Multidisciplinary Sciences
Anatoly Malevanets, P. Andrew Chong, D. Flemming Hansen, Paul Rizk, Yulong Sun, Hong Lin, Ranjith Muhandiram, Avi Chakrabartty, Lewis E. Kay, Julie D. Forman-Kay, Shoshana J. Wodak
SCIENTIFIC REPORTS
(2017)
Article
Biochemistry & Molecular Biology
Meili Liu, Akshaya K. Das, James Lincoff, Sukanya Sasmal, Sara Y. Cheng, Robert M. Vernon, Julie D. Forman-Kay, Teresa Head-Gordon
Summary: This study explores the connection between protein force fields and configurational entropy, proposing a new direction for understanding protein force field development. The research finds that force fields with the largest statistical fluctuations better describe IDPs and IDRs, as well as disorder-to-order transitions.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Hao Cai, Robert M. Vernon, Julie D. Forman-Kay
Summary: Protein phase separation, driven by intrinsically disordered protein regions (IDRs), is an important mechanism of biological organization. LLPhyScore, a new predictor of IDR-driven phase separation, uses sequence-based statistics and a broad set of physical interactions to provide predictions and breakdowns of the contribution from each physical feature. The tool is useful for guiding experiments and hypotheses for protein function, as well as understanding biomolecular condensates.