Review
Biochemistry & Molecular Biology
Jin-Beom Si, Bokyung Kim, Jin Hae Kim
Summary: TTR is a crucial transporter of thyroid hormone and retinol binding protein in human plasma and cerebrospinal fluid, yet it is also known for its amyloidogenic nature leading to various amyloidoses. Research has shown that decreased stability of TTR's native tetrameric conformation is the main cause of these diseases, and recent multidisciplinary investigations have shed light on the mechanistic details of TTR amyloidogenic transformation. Special emphasis has been placed on identifying novel structural features in amyloidogenic species of TTR and discussing the proteolysis-induced fragmentation mechanism that promotes TTR amyloidosis.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Multidisciplinary Sciences
Sarah M. Fantin, Kristine F. Parson, Pramod Yadav, Brock Juliano, Geoffrey C. Li, Charles R. Sanders, Melanie D. Ohi, Brandon T. Ruotolo
Summary: Peripheral myelin protein (PMP22) misfolding is identified as a key factor in various peripheral neuropathies. Mutant forms of PMP22 exhibit differences in stability and propensity to form homodimeric complexes compared to wild-type protein. The formation of PMP22 dimers from destabilized monomers is proposed as a key element in PMP22 mistrafficking, based on combined findings from native ion mobility-mass spectrometry and cellular data.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Multidisciplinary Sciences
Michelle A. E. Anderson, Estela Gonzalez, Joshua X. D. Ang, Lewis Shackleford, Katherine Nevard, Sebald A. N. Verkuijl, Matthew P. P. Edgington, Tim Harvey-Samuel, Luke Alphey
Summary: CRISPR/Cas9-based homing gene drives are a potential new approach to mosquito control. In this study, researchers successfully generated transgenic Ae. aegypti lines expressing Cas9, which significantly biased the inheritance of an sgRNA-expressing element. The sds3G1-Cas9 isolate showed the highest average inheritance, indicating its potential for driving the spread of the targeted element more efficiently.
NATURE COMMUNICATIONS
(2023)
Article
Biochemistry & Molecular Biology
Anna Lizon, Joanna Tisonczyk, Marta Gajewska, Ryszard Drozdz
Summary: The study explores the use of silver nanoparticles (AgNPs) to study the aggregation process of FLC and their propensity to form protein deposits, showing that this technology is helpful in identifying clones of immunoglobulin FLC that tend to aggregate.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Francesca Malagrino, Giuliana Fusco, Valeria Pennacchietti, Angelo Toto, Caterina Nardella, Livia Pagano, Alfonso de Simone, Stefano Gianni
Summary: PDZ domains are common protein-protein interaction modules in the human proteome, often present in multiple repeats. PDZD2 is a protein with six PDZ domains, which undergoes proteolytic cleavage to produce sPDZD2, consisting of a tandem of two PDZ domains, namely PDZ5 and PDZ6. The folding pathway of sPDZD2 involves a transient folding intermediate that may be formed from the denaturation of both PDZ5 and PDZ6 domains. Importantly, this intermediate shows increased affinity for the physiological ligand of sPDZD2 compared to the native state. These findings provide an interesting example of a functionally competent misfolded intermediate.
Review
Cell Biology
Qiao Yi Chen, Ting Wen, Peng Wu, Rui Jia, Ronghua Zhang, Jingxia Dang
Summary: Recent research on exosomes in neurobiology and neurogenerative diseases has shown promising potential for diagnostic and therapeutic applications in ALS. Exosomes are believed to be valuable diagnostic biomarkers and mediators of neuronal degeneration in ALS. However, there are limitations in current isolation and detection methodologies for exosomes in ALS research, despite their therapeutic potential.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Ivana Sirangelo, Clara Iannuzzi
Summary: The study provides an overview of the molecular effects induced by glycation on the amyloid aggregation process of protein models associated with misfolding diseases. Glycation plays a key role in protein folding, kinetics of amyloid formation, and amyloid cytotoxicity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Maurizio Brunori
Summary: The process of protein folding is a spontaneous reversible process leading from an ensemble of disordered structures to the ordered functional protein. In addition to the native and denatured states, proteins can also populate a third state called amyloid, which is characteristic of incurable neurodegenerative disorders.
Article
Biochemistry & Molecular Biology
Surbhi Chaudhary, Asmita Dhiman, Anil Patidar, Himanshu Malhotra, Sharmila Talukdar, Rahul Dilawari, Gaurav Kumar Chaubey, Radheshyam Modanwal, Chaaya Iyengar Raje, Manoj Raje
Summary: Studies have shown that the cytoplasmic glyceraldehyde-3-phosphate dehydrogenase (GAPDH) can prevent protein aggregation, maintain proteins in a soluble state, and interact with cyPrP and httQ-103.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2021)
Article
Biochemistry & Molecular Biology
Keiji Uchiyama, Hideyuki Hara, Junji Chida, Agriani Dini Pasiana, Morikazu Imamura, Tsuyoshi Mori, Hanae Takatsuki, Ryuichiro Atarashi, Suehiro Sakaguchi
Summary: Advanced DMEM may contain anti-prion compounds that reduce PrPSc levels, with ethanolamine identified as having anti-prion activity. Oral administration of ethanolamine delayed the onset of prion disease in mice, suggesting it could be a new anti-prion compound.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Neurosciences
Stefano Thellung, Alessandro Corsaro, Irene Dellacasagrande, Mario Nizzari, Martina Zambito, Tullio Florio
Summary: Transmissible spongiform encephalopathies (TSEs), or prion diseases, are progressive neurodegenerative disorders that affect humans and animals. Current treatments for these diseases are ineffective. The misfolding of prion protein is a key factor in the pathogenesis of TSEs, and alterations in protein homeostasis worsen the severity of the disease. Restoring protein homeostasis pathways through pharmacological interventions may help reduce the burden of misfolded prion protein and improve the condition of patients.
FRONTIERS IN NEUROSCIENCE
(2022)
Editorial Material
Cell Biology
Jeffrey Knupp, Yu-Jie Chen, Anoop Arunagiri, Leena Haataja, Peter Arvan, Billy Tsai
Summary: The study reveals that RTN3 recruits misfolded prohormones for lysosomal degradation through PGRMC1, selectively targeting small oligomers rather than large protein aggregates. This finding suggests that PGRMC1 could be a potential intervention point for diseases caused by ER protein retention.
Article
Biochemistry & Molecular Biology
Priyanka Borah, Airy Sanjeev, Venkata Satish Kumar Mattaparthi
Summary: Parkinson's disease is a common progressive neurodegenerative disorder caused by misfolding and aggregation of alpha-synuclein. Recent research shows that Oleuropein aglycone (OleA) stabilizes the monomeric structure of alpha-synuclein, preventing the formation of toxic aggregates and favoring the growth of stable ones, highlighting a potential therapeutic approach.
JOURNAL OF BIOMOLECULAR STRUCTURE & DYNAMICS
(2021)
Article
Multidisciplinary Sciences
Maxime Belondrade, Simon Nicot, Charly Mayran, Lilian Bruyere-Ostells, Florian Almela, Michele A. Di Bari, Etienne Levavasseur, Joel C. Watts, Chantal Fournier-Wirth, Sylvain Lehmann, Stephane Haik, Romolo Nonno, Daisy Bougard
Summary: This study evaluated the amplification of PrPTSE from human sCJD brain samples in different substrates using PMCA, revealing the potential of bank vole PrP substrate in amplifying all sCJD subtypes. In contrast, PMCA in human PrP substrates led to molecular shifts in PrPTSE, with increased permissiveness of V129 PrP substrate to sCJD prion amplification. The combination of PMCA sensitivities and PrPTSE electrophoretic profiles confirmed the classification of 4 distinct major sCJD prion strains. Additionally, the sensitivity required to detect VV2 sCJD prions in cerebrospinal fluid was achieved.
SCIENTIFIC REPORTS
(2021)
Article
Multidisciplinary Sciences
Kimberly Jia Yi Low, Anandalakshmi Venkatraman, Jodhbir S. Mehta, Konstantin Pervushin
Summary: Protein aggregation and deposition in the form of amyloid fibrils is a characteristic feature of amyloid diseases. While most research focuses on inhibiting amyloid formation, it is equally important to understand the disaggregation mechanisms and their potential therapeutic applications. This review compiles the known mechanisms of amyloid disaggregation and explores the use of disaggregases for treating amyloidosis.
JOURNAL OF ADVANCED RESEARCH
(2022)
Article
Biochemistry & Molecular Biology
F. Kruiswijk, S. C. Hasenfuss, R. Sivapatham, M. P. Baar, D. Putavet, K. A. T. Naipal, N. J. F. van den Broek, W. Kruit, P. J. van der Spek, D. C. van Gent, A. B. Brenkman, J. Campisi, B. M. T. Burgering, J. H. J. Hoeijmakers, P. L. J. de Keizer
Article
Pharmacology & Pharmacy
Edilio Borroni, Bernd Bohrmann, Fiona Grueninger, Eric Prinssen, Stephane Nave, Hansruedi Loetscher, Shankar J. Chinta, Subramanian Rajagopalan, Anand Rane, Almas Siddiqui, Bart Ellenbroek, Juerg Messer, Axel Pahler, Julie K. Andersen, Rene Wyler, Andrea M. Cesura
JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS
(2017)
Article
Cell Biology
Dmitri Leonoudakis, Anand Rane, Suzanne Angeli, Gordon J. Lithgow, Julie K. Andersen, Shankar J. Chinta
MEDIATORS OF INFLAMMATION
(2017)
Article
Neurosciences
Banibrata Das, Subramanian Rajagopalan, Gnanada S. Joshi, Liping Xu, Dan Luo, Julie K. Andersen, Sokol V. Todi, Aloke K. Dutta
Article
Multidisciplinary Sciences
Mark Lucanic, W. Todd Plummer, Esteban Chen, Jailynn Harke, Anna C. Foulger, Brian Onken, Anna L. Coleman-Hulbert, Kathleen J. Dumas, Suzhen Guo, Erik Johnson, Dipa Bhaumik, Jian Xue, Anna B. Crist, Michael P. Presley, Girish Harinath, Christine A. Sedore, Manish Chamoli, Shaunak Kamat, Michelle K. Chen, Suzanne Angeli, Christina Chang, John H. Willis, Daniel Edgar, Mary Anne Royal, Elizabeth A. Chao, Shobhna Patel, Theo Garrett, Carolina Ibanez-Ventoso, June Hope, Jason L. Kish, Max Guo, Gordon J. Lithgow, Monica Driscoll, Patrick C. Phillips
NATURE COMMUNICATIONS
(2017)
Review
Clinical Neurology
Manish Chamoli, Shankar J. Chinta, Julie K. Andersen
JOURNAL OF NEURAL TRANSMISSION
(2018)
Article
Neurosciences
Anand Rane, Subramanian Rajagopalan, Manuj Ahuja, Bobby Thomas, Shankar J. Chinta, Julie K. Andersen
Article
Toxicology
Georgia Woods, Julie K. Andersen
TOXICOLOGICAL SCIENCES
(2018)
Article
Cell Biology
Shankar J. Chinta, Georgia Woods, Marco Demaria, Anand Rane, Ying Zou, Amanda McQuade, Subramanian Rajagopalan, Chandani Limbad, David T. Madden, Judith Campisi, Julie K. Andersen
Article
Multidisciplinary Sciences
Xueshu Xie, Manish Chamoli, Dipa Bhaumik, Renuka Sivapatham, Suzanne Angeli, Julie K. Andersen, Gordon J. Lithgow, Birgit Schilling
JOVE-JOURNAL OF VISUALIZED EXPERIMENTS
(2020)
Article
Biology
Suzanne Angeli, Anna Foulger, Manish Chamoli, Tanuja Harshani Peiris, Akos Gerencser, Azar Asadi Shahmirzadi, Julie Andersen, Gordon Lithgow
Summary: Mitochondrial activity plays a crucial role in determining aging rate and the onset of chronic diseases. Research suggests that the destabilization of OSCP in the inner mitochondrial membrane may promote aging by initiating the mPTP. Inhibition of mPTP can suppress UPRmt and restore normal lifespan, indicating a potential protective role of UPRmt inhibition in aging and age-related diseases.
Article
Cell Biology
Jae-Hyun Yang, Christopher A. Petty, Thomas Dixon-McDougall, Maria Vina Lopez, Alexander Tyshkovskiy, Sun Maybury-Lewis, Xiao Tian, Nabilah Ibrahim, Zhili Chen, Patrick T. Griffin, Matthew Arnold, Jien Li, Oswaldo A. Martinez, Alexander Behn, Ryan Rogers-Hammond, Suzanne Angeli, Vadim N. Gladyshev, David A. Sinclair
Summary: A hallmark of eukaryotic aging is a loss of epigenetic information, a process that can be reversed. By ectopically inducing the Yamanaka factors OCT4, SOX2, and KLF4 (OSK) in mammals, we can restore youthful characteristics without changing cellular identity. Through high-throughput cell-based assays, we have identified six chemical cocktails that can reverse cellular aging and rejuvenate human cells without altering the genome.
Article
Cell & Tissue Engineering
Samantha Haller, Subir Kapuria, Rebeccah R. Riley, Monique N. O'Leary, Katherine H. Schreiber, Julie K. Andersen, Simon Melov, Jianwen Que, Thomas A. Rando, Jason Rock, Brian K. Kennedy, Joseph T. Rodgers, Heinrich Jasper
Meeting Abstract
Cell & Tissue Engineering
B. Ahmadian Baghbaderani, A. Syama, R. Sivapatham, Y. Pei, O. Mukherjee, X. Tian, H. Tran, L. Menendez, T. Fellner, X. Zeng, M. Rao