Article
Chemistry, Analytical
Xin Shu, Qing-Qing Liao, Shang-Tong Li, Lu Liu, Xiajun Zhang, Lianqi Zhou, Long Zhang, Irene Coin, Lei Wang, Haifan Wu, Bing Yang
Summary: Post-translational modification of proteins by Ubiquitin (Ub) and Ubiquitin-like proteins (Ubls) can be reversed by deconjugating enzymes. Developing cell-permeable Ub/Ubl probes for live cell applications is challenging. Here, genetically encoded cross-linkable Ub/Ubl probes were developed to investigate deconjugating enzyme activity in physiological environments.
ANALYTICAL CHEMISTRY
(2023)
Article
Critical Care Medicine
Sophie Stukas, Jasmine Gill, Jennifer Cooper, Lise Belanger, Leanna Ritchie, Angela Tsang, Kevin Dong, Femke Streijger, John Street, Scott Paquette, Tamir Ailon, Nicolas Dea, Raphaele Charest-Morin, Charles G. Fisher, Sanjay Dhall, Jean-Marc Mac-Thiong, Jefferson R. Wilson, Christopher Bailey, Sean Christie, Marcel F. Dvorak, Cheryl Wellington, Brian K. Kwon
Summary: The study found that CSF UCH-L1 levels were significantly elevated in acute SCI patients, could distinguish between different AIS grades at 24 hours post-injury, and predict who would remain motor complete at 6 months post-injury. However, serum UCH-L1 levels were not informative about injury severity or outcome.
JOURNAL OF NEUROTRAUMA
(2021)
Article
Rheumatology
Y. Guo, X. Li, R. Li, Y. Li, Z. Wang, H. Liu, S. Cao, Ru Li, Y. Zhao, Q. Wang, X. Sun
Summary: This study investigates the diagnostic potential of serum autoantibodies against different UCH-L1 epitopes in NPSLE. The results show that the autoantibody against amino acid 58 to 69 of UCH-L1 (UCH58-69) has the highest diagnostic power in distinguishing NPSLE patients from SLE patients without neuro-psychiatric symptoms. Moreover, increased serum anti-UCH58-69 levels are associated with disease severity in SLE patients.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2022)
Article
Multidisciplinary Sciences
Julia Reichelt, Wiebke Sachs, Sarah Froembling, Julia Fehlert, Maja Studencka-Turski, Anna Betz, Desiree Loreth, Lukas Blume, Susanne Witt, Sandra Pohl, Johannes Brand, Maire Czesla, Jan Knop, Bogdan I. Florea, Stephanie Zielinski, Marlies Sachs, Elion Hoxha, Irm Hermans-Borgmeyer, Gunther Zahner, Thorsten Wiech, Elke Krueger, Catherine Meyer-Schwesinger
Summary: In membranous nephropathy, autoantibodies target podocytes of the kidney filter, resulting in injury. The authors found that the disturbances in protein stability and proteinuria are related to abnormal interactions between non-functional UCH-L1 enzyme and the proteasome, reducing its capacity. The mechanistic significance of the ubiquitin proteasome system (UPS) in a kidney autoimmune environment is not well understood.
NATURE COMMUNICATIONS
(2023)
Article
Cell Biology
Shuo Tian, Shouheng Jin, Yaoxing Wu, Tao Liu, Man Luo, Jiayu Ou, Weihong Xie, Jun Cui
Summary: This study identified previously unrecognized roles of deubiquitinating enzymes in modulating autophagy and provided insights into the critical roles of reversible ubiquitination in modifying ATG proteins.
Article
Biochemistry & Molecular Biology
Chipeng Guo, Lizhu Liang, Jingbin Zheng, Yang Xie, Xiaonan Qiu, Guozhen Tan, Jingang Huang, Liangchun Wang
Summary: In this study, it was found that UCHL1 was significantly upregulated in keloid fibroblasts and played a role in the pathological process of keloid by promoting collagen I and alpha-SMA expression. UCHL1 expression was also found to be promoted by M2 macrophages via TGF-beta 1. These findings expand our understanding of the pathogenesis of keloid and provide potential therapeutic targets for the disease.
Article
Biochemistry & Molecular Biology
Thoa Kim Truong Huynh, Trinh Thi Thu Mai, Man Anh Huynh, Hideki Yoshida, Masamitsu Yamaguchi, Thao Thi Phuong Dang
Summary: This study demonstrates the impact of UCH-L1 deficiency on motor neuron health using a Drosophila model, highlighting the crucial role of UCH-L1 in neuronal health.
ANTIOXIDANTS & REDOX SIGNALING
(2022)
Article
Biotechnology & Applied Microbiology
Manibarathi Vaithiyanathan, Yongmei Yu, Alireza Rahnama, Jacob H. Pettigrew, Nora Safa, Dong Liu, Ted J. Gauthier, Z. Elizabeth Floyd, Adam T. Melvin
Summary: Deubiquitinating enzyme (DUB)-targeted therapeutics have shown promise as alternative cancer treatments, with the botanical extract PMI-5011 found to positively regulate DUB activity in two multiple myeloma cell lines. Additionally, bioactive compounds in PMI-5011 showed similar effects on DUB activity, suggesting therapeutic potential in cancer treatment.
BIOCHEMICAL ENGINEERING JOURNAL
(2021)
Review
Cell Biology
Donghui Yang, Qizhong Lu, Sha Peng, Jinlian Hua
Summary: This study summarizes the important function of UCHL1 in controlling mammalian oocyte development, regulating spermatogenesis, and inhibiting polyspermy. It highlights the essential role of UCHL1 balance in maintaining reproductive cellular and tissue homeostasis.
CELL PROLIFERATION
(2023)
Article
Clinical Neurology
Ana M. Castano-Leon, Cristina Sanchez Carabias, Amaya Hilario, Ana Ramos, Blanca Navarro-Main, Igor Paredes, Pablo M. Munarriz, Irene Panero, Carla Eiriz Fernandez, Daniel Garcia-Perez, Luis Miguel Moreno-Gomez, Olga Esteban-Sinovas, Guillermo Garcia Posadas, Pedro A. Gomez, Alfonso Lagares
Summary: This study explores the potential role of serum biomarkers in assessing traumatic axonal injury (TAI). By extracting blood and performing imaging assessments on traumatic brain injury (TBI) patients, it was found that the concentrations of biomarkers were significantly higher in TBI patients compared to the control group. Among these biomarkers, UCH-L1 and NfL appear to be more specific markers for detecting TAI. Furthermore, the combination of NfL and the fractional anisotropy (FA) of the corpus callosum (CC) can help predict long-term outcomes.
JOURNAL OF NEUROSURGERY
(2023)
Article
Medical Laboratory Technology
Charlotte Oris, Jean-Baptiste Bouillon-Minois, Samy Kahouadji, Bruno Pereira, Gabriel Dhaiby, Valentin Bailly Defrance, Julie Durif, Jeannot Schmidt, Fares Moustafa, Damien Bouvier, Vincent Sapin
Summary: Measuring plasma GFAP and UCH-L1 levels is useful in targeting and optimizing the reduction of CT scans in mTBI patients.
CLINICAL CHEMISTRY AND LABORATORY MEDICINE
(2023)
Review
Cell Biology
Wenjing Zhu, Dandan Zheng, Dandan Wang, Lehe Yang, Chengguang Zhao, Xiaoying Huang
Summary: The article summarizes the roles of the deubiquitinating enzyme USP25 in disease progression. Previous studies have mainly focused on its role in antiviral immunity and neurodegenerative diseases, but recent research has gradually revealed the mechanisms of action of USP25 in cancer and other diseases.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Neurosciences
Zhiping Mi, Hao Liu, Marie E. Rose, Jie Ma, Daniel P. Reay, Xiecheng Ma, Jeremy J. Henchir, C. Edward Dixon, Steven H. Graham
Summary: The study found that mutating cysteine to alanine at site 152 of UCHL1 can reduce protein ubiquitination, decrease activation of autophagy markers, improve abnormal axons and cell death, and enhance motor and cognitive function after traumatic brain injury. This research provides a new target for future therapeutic approaches in TBI.
Article
Pharmacology & Pharmacy
M. ZHANG, M. -Y. CHEN, S. -L. WANG, X. -M. DING, R. YANG, J. LI, G. -H. JIANG
Summary: This study aimed to investigate the relationship between serum ubiquitin c-terminal hydrolase (Uch-L1) and cognition and brain energy metabolism, and whether it can be used as an early blood marker for Alzheimer's disease. The results showed a significant correlation between serum Uch-L1 concentration and cognitive ability and brain energy metabolism, suggesting its potential as a blood marker for extensive dementia screening in the future.
EUROPEAN REVIEW FOR MEDICAL AND PHARMACOLOGICAL SCIENCES
(2022)
Review
Biochemistry & Molecular Biology
Eliona Tsefou, Robin Ketteler
Summary: Quality control of mitochondria is crucial for cell survival, and defects can lead to severe diseases. Mitophagy plays a key role in removing damaged mitochondria and enhancing mitophagy has the potential to improve cell survival, neuronal function, and lifespan. Deubiquitinating enzymes are important regulators of mitophagy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Oncology
Mark Bustoros, Romanos Sklavenitis-Pistofidis, Jihye Park, Robert Redd, Benny Zhitomirsky, Andrew J. Dunford, Karma Salem, Yu-Tzu Tai, Shankara Anand, Tarek H. Mouhieddine, Selina J. Chavda, Cody Boehner, Liudmila Elagina, Carl Jannes Neuse, Justin Cha, Mahshid Rahmat, Amaro Taylor-Weiner, Eliezer Van Allen, Shaji Kumar, Efstathis Kastritis, Ignaty Leshchiner, Elizabeth A. Morgan, Jacob Laubach, Tineke Casneuf, Paul Richardson, Nikhil C. Munshi, Kenneth C. Anderson, Lorenzo Trippa, Francois Aguet, Chip Stewart, Meletios-Athanasios Dimopoulos, Kwee Yong, P. Leif Bergsagel, Salomon Manier, Gad Getz, Irene M. Ghobrial
JOURNAL OF CLINICAL ONCOLOGY
(2020)
Article
Oncology
Cecilia Bonolo de Campos, Nathalie Meurice, Joachim L. Petit, Alysia N. Polito, Yuan Xiao Zhu, Panwen Wang, Laura A. Bruins, Xuewei Wang, Ilsel D. Lopez Armenta, Susie A. Darvish, Greg J. Ahmann, Kimberly J. Henderson, Shulan Tian, Jonas J. Kruse, William M. Stewart, Jeremy T. Larsen, Craig B. Reeder, David Dingli, Prashant Kapoor, Shaji K. Kumar, Rafael Fonseca, P. Leif Bergsagel, Esteban Braggio, A. Keith Stewart
BLOOD CANCER JOURNAL
(2020)
Letter
Oncology
Linda B. Baughn, Zhuo Li, Kathryn Pearce, Celine M. Vachon, Mei-Yin Polley, Jonathan Keats, Eran Elhaik, Michael Baird, Terry Therneau, James R. Cerhan, P. Leif Bergsagel, Angela Dispenzieri, S. Vincent Rajkumar, Yan W. Asmann, Shaji Kumar
BLOOD CANCER JOURNAL
(2020)
Article
Hematology
Zhi Wen, Adhithi Rajagopalan, Evan D. Flietner, Grant Yun, Marta Chesi, Quinlan Furumo, Robert T. Burns, Athanasios Papadas, Erik A. Ranheim, Adam C. Pagenkopf, Zachary T. Morrow, Remington Finn, Yun Zhou, Shuyi Li, Xiaona You, Jeffrey Jensen, Mei Yu, Alexander Cicala, James Menting, Constantine S. Mitsiades, Natalie S. Callander, P. Leif Bergsagel, Demin Wang, Fotis Asimakopoulos, Jing Zhang
Summary: NRAS Q61 mutations are prevalent in advanced/relapsed multiple myeloma, with poor patient outcomes. A novel MM model was generated in mice, exhibiting characteristics similar to human advanced/high-risk MM. MEK inhibition-based therapies significantly prolonged survival in advanced-stage MM mice, suggesting potential for future clinical application.
Article
Cell Biology
Zongwei Li, Huan Liu, Jin He, Zhiqiang Wang, Zheng Yin, Gichun You, Zhiming Wang, Richard E. Davis, Pei Lin, Leif Bergsagel, Elisabet E. Manasanch, Stephen T. C. Wong, Nestor F. Esnaola, Jenny C. Chang, Robert Z. Orlowski, Qing Yi, Jing Yang
Summary: The study indicates that acetyl-CoA synthetase 2 (ACSS2) plays an important role in obesity-related myeloma progression, interacting with oncoprotein interferon regulatory factor 4 (IRF4) to enhance tumor growth. Overexpression of ACSS2 contributes to IRF4 stability and gene transcription, reducing myeloma growth in an obese mouse model.
Article
Oncology
Yan W. Asmann, Kaushal Parikh, P. Leif Bergsagel, Haidong Dong, Alex A. Adjei, Mitesh J. Borad, Aaron S. Mansfield
Summary: The recent FDA approval of TMB-H status as a biomarker for PD-1 inhibitor treatment highlights the importance of accurate assessment of patient-specific TMB. Research shows that using public databases for TMB estimation without patient-paired germline sequencing may introduce racial bias, with TMB being more inflated in Black patients compared to White patients.
NPJ PRECISION ONCOLOGY
(2021)
Article
Medicine, General & Internal
Eli Muchtar, Angela Dispenzieri, Morie A. Gertz, Shaji K. Kumar, Francis K. Buadi, Nelson Leung, Martha Q. Lacy, David Dingli, Sikander Ailawadhi, P. Leif Bergsagel, Rafael Fonseca, Suzanne R. Hayman, Prashant Kapoor, Martha Grogan, Omar F. Abou Ezzeddine, Julie L. Rosenthal, Michelle Mauermann, Mustaqueem Siddiqui, Wilson Gonsalves, Taxiarchis Kourelis, Jeremy T. Larsen, Craig B. Reeder, Rahma Warsame, Ronald S. Go, David L. Murray, Ellen D. McPhail, Surendra Dasari, Dragan Jevremovic, Robert A. Kyle, Yi Lin, John A. Lust, Stephen J. Russell, Yi Lisa Hwa, Amie L. Fonder, Miriam A. Hobbs, S. Vincent Rajkumar, Vivek Roy, Taimur Sher
Summary: AL amyloidosis is a clonal plasma cell disorder that can lead to organ failure. Early recognition, anti-plasma cell therapy, and supportive care are important for improving patient survival.
MAYO CLINIC PROCEEDINGS
(2021)
Article
Oncology
Erin W. Meermeier, Seth J. Welsh, Meaghen E. Sharik, Megan T. Du, Victoria M. Garbitt, Daniel L. Riggs, Chang-Xin Shi, Caleb K. Stein, Marco Bergsagel, Bryant Chau, Matthew L. Wheeler, Natalie Bezman, Feng Wang, Pavel Strop, P. Leif Bergsagel, Marta Chesi
Summary: A study on BCMA/CD3-targeting bispecific antibodies (BsAb) in mouse models of multiple myeloma revealed that the BsAb was safe and effective in some cases but not in those with high tumor burden, with combination with pomalidomide showing improved efficacy but limited survival due to toxicity and T-cell exhaustion. However, combining with cyclophosphamide was safe and prevented T-cell exhaustion, promoting long-term disease control.
BLOOD CANCER DISCOVERY
(2021)
Review
Oncology
Philippe Moreau, Shaji K. Kumar, Jesus San Miguel, Faith Davies, Elena Zamagni, Nizar Bahlis, Heinz Ludwig, Joseph Mikhael, Evangelos Terpos, Fredrik Schjesvold, Thomas Martin, Kwee Yong, Brian G. M. Durie, Thierry Facon, Artur Jurczyszyn, Surbhi Sidana, Noopur Raje, Niels van de Donk, Sagar Lonial, Michele Cavo, Sigurdur Y. Kristinsson, Suzanne Lentzsch, Roman Hajek, Kenneth C. Anderson, Cristina Joao, Hermann Einsele, Pieter Sonneveld, Monika Engelhardt, Rafael Fonseca, Annette Vangsted, Katja Weisel, Rachid Baz, Vania Hungria, Jesus G. Berdeja, Fernando Leal da Costa, Angelo Maiolino, Anders Waage, David H. Vesole, Enrique M. Ocio, Hang Quach, Christoph Driessen, Joan Blade, Xavier Leleu, Eloisa Riva, Peter Leif Bergsagel, Jian Hou, Wee Joo Chng, Ulf-Henrik Mellqvist, Dominik Dytfeld, Jean-Luc Harousseau, Hartmut Goldschmidt, Jacob Laubach, Nikhil C. Munshi, Francesca Gay, Meral Beksac, Luciano J. Costa, Martin Kaiser, Parameswaran Hari, Mario Boccadoro, Saad Z. Usmani, Sonja Zweegman, Sarah Holstein, Orhan Sezer, Simon Harrison, Hareth Nahi, Gordon Cook, Maria-Victoria Mateos, S. Vincent Rajkumar, Meletios A. Dimopoulos, Paul G. Richardson
Summary: This policy review presents clinical practice recommendations for the treatment of relapsed and refractory multiple myeloma, integrating drug access issues for patients in different income settings to improve patient outcomes in real-world practice.
Meeting Abstract
Hematology
Neeraj Sharma, James B. Smadbeck, Nadine Abdallah, Kathryn E. Pearce, Yan Asmann, Jess Peterson, Rhett P. Ketterling, Patricia Greipp, P. Leif Bergsagel, S. Vincent Rajkumar, Shaji K. Kumar, Linda B. Baughn
Meeting Abstract
Hematology
Christopher T. Su, Liying Chen, Jason Chen, Brian Parkin, Avery Polk, Malathi Kandarpa, Craig E. Cole, Erica Campagnaro, Josh Vo, Dan Robinson, Yi-Mi Wu, Moshe Talpaz, Jennifer Yesil, Daniel Auclair, P. Leif Bergsagel, Arul Chinnaiyan, Veerabhadran Baladandayuthapani, J. Christine Ye
Meeting Abstract
Hematology
Joselle Cook, Kah-Whye Peng, Brenda F. Ginos, Amylou C. Dueck, Marissa Giers, Nandakumar Packiriswamy, Beth Brunton, Mrinal M. Patnaik, Thomas E. Witzig, Francis K. Buadi, Angela Dispenzieri, Morie A. Gertz, Ronald S. Go, Suzanne R. Hayman, John A. Lust, David Dingli, S. Vincent Rajkumar, Nelson Leung, Rahma M. Warsame, Wilson I. Gonsalves, Taxiarchis Kourelis, Eli Muchtar, P. Leif Bergsagel, Stephen Broski, Prashant Kapoor, Robert A. Kyle, Mustaqeem A. Siddiqui, Amie Fonder, Miriam A. Hobbs, Yi L. Hwa, Yi Lin, Shaji K. Kumar, Stephen J. Russell, Martha Q. Lacy
Meeting Abstract
Hematology
Miguel Gonzalez-Velez, Mariano Arribas, Heidi E. Kosiorek, Richard Butterfield, Carlo Guerrero, Luke Mountjoy, Jeremy Larsen, Craig B. Reeder, P. Leif Bergsagel, A. Keith Stewart, Rafael Fonseca
Meeting Abstract
Hematology
Miguel Gonzalez-Velez, Mariano Arribas, Heidi E. Kosiorek, Richard Butterfield, Carlo Guerrero, Luke Mountjoy, Jeremy Larsen, Craig B. Reeder, P. Leif Bergsagel, A. Keith Stewart, Rafael Fonseca
Meeting Abstract
Hematology
Taxiarchis Kourelis, Sikander Ailawadhi, Dan T. Vogl, Dennis Cooper, Tyler D. Ames, Christina Y. Yim, Matthew R. Price, Jose J. Jimeno, P. Leif Bergsagel