Article
Cell Biology
Yvette Akwa, Chiara Di Malta, Fatima Zallo, Elise Gondard, Adele Lunati, Lara Z. Diaz-de-Grenu, Angela Zampelli, Anne Boiret, Sara Santamaria, Maialen Martinez-Preciado, Katia Cortese, Jeffrey H. Kordower, Carlos Matute, Andres M. Lozano, Estibaliz Capetillo-Zarate, Thomas Vaccari, Carmine Settembre, Etienne E. Baulieu, Davide Tampellini
Summary: Synaptic stimulation promotes TFEB-mediated clearance of pathological MAPT/Tau, which provides neuroprotection. Deep brain stimulation activates TFEB and reduces MAPT/Tau levels in Parkinson disease patients, leading to neuroprotection.
Article
Biochemistry & Molecular Biology
Chang-Heng Hsieh, Chia-Cheng Chou, Ya-Ching Fang, Po-Hao Hsu, Yi-Hung Chiu, Chi-Sheng Yang, Guey-Mei Jow, Chih-Yung Tang, Chung-Jiuan Jeng
Summary: Endogenous 14-3-3 proteins play a chaperone-like role in regulating the stability of Eag1 protein and modulators of 14-3-3 have therapeutic potential in correcting protein expression of disease-causing Eag1 mutants.
CELL AND BIOSCIENCE
(2023)
Article
Biochemistry & Molecular Biology
Pingting Liu, Md Razaul Karim, Ana Covelo, Yuan Yue, Michael K. K. Lee, Wensheng Lin
Summary: The unfolded protein response (UPR) is a major mechanism for maintaining cellular proteostasis. It is involved in regulating neuron viability and function in neurodegenerative diseases, but its role is controversial. While evidence suggests UPR activation in neurons under normal conditions, deficiency of individual UPR branches has no major effect on brain neurons in animals. To determine the physiological role of UPR, mice with double deletion of PERK and ATF6α in neurons were generated. The results showed that UPR is required for maintaining neuronal proteostasis and the viability and function of neurons in the hippocampus of adult mice.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Multidisciplinary Sciences
Yuchen Feng, Aileen R. Ariosa, Ying Yang, Zehan Hu, Joern Dengjel, Daniel J. Klionsky
Summary: Macroautophagy/autophagy is a crucial molecular process for maintaining cellular homeostasis, recycling cytoplasmic materials, and responding to stress conditions. The posttranslational modification of Atg9, including ubiquitination and proteasome-dependent degradation, regulates the level of autophagy in cells.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Cell Biology
Heather Tsong, Erika L. F. Holzbaur, Andrea K. H. Stavoe
Summary: Misregulation of neuronal autophagy is involved in age-related neurodegenerative diseases. Aging leads to a decrease in autophagosome formation rate and density along the axon, but an increase in autophagic vesicle maturation. Late aging results in a significant loss of acidified vesicles in the distal axon, while the efficient transport of autophagic vesicles in both proximal and distal axons is maintained during early aging. These findings suggest that aging differentially impacts distinct aspects of neuronal autophagy.
Article
Chemistry, Medicinal
Haiyu Zhang, Xinmei Xie, Junzhuo Shi, Qian Zhao, Dongmei Luo, Jiaojiao Hao, Yunfeng Zhou, Guang Han, Xiaobin Pang
Summary: The present study confirmed the cognition-enhancing effect of 3, 14, 19-Triacetyl andrographolide (ADA) on learning and memory deficits in 3 x Tg-AD mice. ADA improves synaptic plasticity and cognitive function mainly through inducing autophagy and promoting the degradation process.
PHYTOTHERAPY RESEARCH
(2023)
Article
Endocrinology & Metabolism
Wuxue Peng, Changhong Tan, Lijuan Mo, Jin Jiang, Wen Zhou, Juncong Du, Xuan Zhou, Xi Liu, Lifen Chen
Summary: Neurons rely on GLUT3 to uptake glucose for energy production, playing a crucial role in neuronal energy metabolism, brain function, and development. Regulation of GLUT3 by various factors may contribute to the pathogenesis and progression of neurological diseases, making it a potential therapeutic target.
METABOLISM-CLINICAL AND EXPERIMENTAL
(2021)
Article
Biochemistry & Molecular Biology
Enrico Castroflorio, Joery den Hoed, Daria Svistunova, Mattea J. Finelli, Alberto Cebrian-Serrano, Silvia Corrochano, Andrew R. Bassett, Benjamin Davies, Peter L. Oliver
Summary: Members of the Tre2/Bub2/Cdc16 (TBC), lysin motif (LysM), domain catalytic (TLDc) protein family are associated with neurodevelopmental disorders, including NCOA7 linked to autism. NCOA7 is shown to regulate the function of the vacuolar (V)-ATPase in the brain, with its deletion resulting in neuronal developmental defects and lysosomal dysfunction. Behavioral assessment of Ncoa7 deletion mice revealed anxiety and social defects, indicating the importance of NCOA7 in neurodevelopment.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Article
Neurosciences
Eric N. Moca, Daniela Lecca, Keenan T. Hope, Fanny Etienne, Ari W. Schaler, Katherine Espinoza, Megan S. Chappell, Daniel T. Gray, David Tweedie, Shanaya Sidhu, Lindsay Masukawa, Hannah Sitoy, Rose Mathew, Daniel R. Saban, Nigel H. Greig, Lindsay M. De Biase
Summary: During aging, microglia display different properties and interactions with synapses, affecting nearby neurons. Our study reveals regional differences in microglial response to aging and highlights the importance of lysosome status in microglial aging.
JOURNAL OF NEUROSCIENCE
(2022)
Article
Neurosciences
Chan Choo Yap, Laura Digilio, Lloyd P. McMahon, Tuanlao Wang, Bettina Winckler
Summary: In all cell types, endocytosed cargo is transported along a set of endosomal compartments, from early endosomes (EEs) via late endosomes (LEs) to lysosomes which are critical for degradation of proteins. Rab7 plays a key role in the maturation, motility, and fusion of endosomes with lysosomes. The study demonstrates that dynein is responsible for the retrograde transport of late endosomes in dendrites.
JOURNAL OF NEUROSCIENCE
(2022)
Article
Cell Biology
Yuchun Liu, Yao Sun, Shaoming Han, Yanan Guo, Qingnan Tian, Qiang Ma, Shoutao Zhang
Summary: This study found that CHIP positively regulates NF-kappa B signaling by targeting TAK1 and enhancing its K63-linked ubiquitination, playing a crucial role in neurological disorders.
CELL DEATH DISCOVERY
(2021)
Article
Multidisciplinary Sciences
Zhaokai Li, Xiaoqiang Huang, Mohan Li, Eugene Chen, Zhong Wang, Liu Liu
Summary: This study reports a phosphorylation-induced, ubiquitin-proteasome-system-mediated targeted protein degradation strategy that allows specific degradation of 14-3-3-binding phosphoproteins. This strategy can serve as a universal degrader for studying the functions of 14-3-3-binding phosphoproteins.
Article
Biochemistry & Molecular Biology
Hyunbum Jeon, Yeo Jin Kim, Su-Kyeong Hwang, Jinsoo Seo, Ji Young Mun
Summary: L-serine can serve as a neuroprotective agent by restoring lysosomal function, providing protection against propionic acid-induced neuronal toxicity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Chemistry, Multidisciplinary
Bente A. Somsen, Fenna W. B. Craenmehr, Wei-Hong W. Liu, Auke A. Koops, Marloes A. M. Pennings, Emira J. Visser, Christian Ottmann, Peter J. Cossar, Luc Brunsveld
Summary: Molecular glues represent a novel approach in drug discovery, however, the targeted stabilization of protein complexes is still challenging due to a lack of drug design rules. This study demonstrates the successful development of a peptide-based molecular glue that selectively stabilizes the 14-3-3/ChREBP protein-protein interaction by utilizing the functional mapping of hotspots.
Review
Biochemistry & Molecular Biology
Jing Liu, Lijuan Wang, Hua He, Yueying Liu, Yiqun Jiang, Jinfeng Yang
Summary: Chaperone-mediated autophagy (CMA) is a process that degrades proteins labeled with KFERQ-like motifs within cells to terminate their functioning. CMA plays a crucial role in cell proliferation and apoptosis. Its relationship with cancer is complex, with contradictory effects in cancer cells and untransformed cells, leading to interest in regulating CMA activity for cancer therapy.
Article
Biochemistry & Molecular Biology
Joao R. Gomes, Ines Cabrito, Hugo R. Soares, Susete Costelha, Anabela Teixeira, Angela Wittelsberger, Catelijne Stortelers, Peter Vanlandschoot, Maria J. Saraiva
JOURNAL OF NEUROCHEMISTRY
(2018)
Article
Biochemistry & Molecular Biology
Joao R. Gomes, Zsuzsa Sarkany, Anabela Teixeira, Renata Nogueira, Ines Cabrito, Hugo Soares, Angela Wittelsberger, Catelijne Stortelers, Sandra Macedo-Ribeiro, Peter Vanlandschoot, Maria J. Saraiva
ACS CHEMICAL NEUROSCIENCE
(2019)
Article
Biochemistry & Molecular Biology
Merrill D. Benson, Joel N. Buxbaum, David S. Eisenberg, Giampaolo Merlini, Maria J. M. Saraiva, Yoshiki Sekijima, Jean D. Sipe, Per Westermark
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2018)
Review
Biochemistry & Molecular Biology
Nelson Ferreira, Maria Joao Saraiva, Maria Rosario Almeida
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2019)
Article
Biochemistry & Molecular Biology
Luis F. Maia, Aleksandra Maceski, Isabel Conceicao, Laura Obici, Rui Magalhaes, Andreas Cortese, David Leppert, Giampaolo Merlini, Jens Kuhle, Maria Joao Saraiva
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2020)
Article
Oncology
Christoph Niemietz, Filipa Bezerra, Maria Rosario Almeida, Shuling Guo, Brett P. Monia, Maria Joao Saraiva, Paula Schuetz, Hartmut H-J Schmidt, Andree Zibert
EXPERIMENTAL CELL RESEARCH
(2020)
Review
Neurosciences
Filipa Bezerra, Maria Joao Saraiva, Maria Rosario Almeida
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2020)
Article
Immunology
Joao Moreira, Susete Costelha, Margarida Saraiva, Maria Joao Saraiva
Summary: Inflammation is a key feature of neurodegenerative disorders like ATTRv. Compared to wild type animals, chemokine expression is significantly downregulated in the peripheral nervous system of a mouse model of the disease, possibly due to a mechanism that leads to the inactivation of TTR activation in patients.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Filipa Bezerra, Christoph Niemietz, Hartmut H. J. Schmidt, Andree Zibert, Shuling Guo, Brett P. Monia, Paula Goncalves, Maria Joao Saraiva, Maria Rosario Almeida
Summary: The SerpinA1 plays an important role in regulating the proteolysis and aggregation of transthyretin. Downregulation of SerpinA1 leads to increased TTR levels in mice plasma and deposition in the cardiac tissue.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Multidisciplinary Sciences
Aubin Michalon, Andreas Hagenbuch, Christian Huy, Evita Varela, Benoit Combaluzier, Thibaud Damy, Ole B. Suhr, Maria J. Saraiva, Christoph Hock, Roger M. Nitsch, Jan Grimm
Summary: Through analyzing memory B cell repertoires of healthy elderly, researchers developed a selective human antibody that removes cardiac amyloid by recruiting phagocytic immune cells.
NATURE COMMUNICATIONS
(2021)
Article
Clinical Neurology
Joao R. Gomes, Andrea Lobo, Renata Nogueira, Ana F. Terceiro, Susete Costelha, Igor M. Lopes, Ana Magalhaes, Teresa Summavielle, Maria J. Saraiva
BRAIN COMMUNICATIONS
(2020)
Article
Biochemistry & Molecular Biology
Merrill D. Benson, Joel N. Buxbaum, David S. Eisenberg, Giampaolo Merlini, Maria J. M. Saraiva, Yoshiki Sekijima, Jean D. Sipe, Per Westermark
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2020)
Meeting Abstract
Biochemistry & Molecular Biology
Filipa Bezerra, Carlos J. V. Simoes, Joao M. Beirao, Maria Joao Saraiva, Rui M. M. Brito, Maria Rosario Almeida
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2019)
Meeting Abstract
Biochemistry & Molecular Biology
Joao Moreira, Margarida Saraiva, Maria Joao Saraiva
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2019)
Meeting Abstract
Biochemistry & Molecular Biology
Carlos J. V. Simoes, Zaida L. Almeida, Ana L. Cardoso, Filipa Bezerra, Maria R. Almeida, Joao Beirao, Teresa M. V. D. Pinho e Melo, Maria J. Saraiva, Rui M. M. Brito
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2019)