Results 1 to 10 of about 3,775 (171)

Shared and distinct mechanisms of UBA1 inactivation across different diseases

EMBO Journal, 2023
Most cellular ubiquitin signaling is initiated by UBA1, which activates and transfers ubiquitin to tens of E2 enzymes. Clonally acquired UBA1 missense mutations cause an inflammatory-hematologic overlap disease called VEXAS (vacuoles, E1, X-linked ...
Jason C Collins, Samuel J Magaziner, Maya English   +2 more
exaly   +5 more sources

Auranofin targets UBA1 and enhances UBA1 activity by facilitating ubiquitin trans-thioesterification to E2 ubiquitin-conjugating enzymes

Nature Communications, 2023
UBA1 is the primary E1 ubiquitin-activating enzyme responsible for generation of activated ubiquitin required for ubiquitination, a process that regulates stability and function of numerous proteins.
Wenjing Yan, Yongwang Zhong, Xin Hu, Tuan Xu, Yinghua Zhang, Stephen Kales, Yanyan Qu, Daniel C. Talley, Bolormaa Baljinnyam, Christopher A. LeClair, Anton Simeonov, Brian M. Polster, Ruili Huang, Yihong Ye, Ganesha Rai, Mark J. Henderson, Dingyin Tao, Shengyun Fang   +17 more
doaj   +3 more sources

UBA1 promotes cardiac hypertrophy by suppressing autophagy via targeting ATG5 for ubiquitination [PDF]

Cell Communication and Signaling
Background Pathological cardiac hypertrophy frequently leads to heart failure (HF). UBA1, the key E1 ubiquitin-activating enzyme, initiates ubiquitin-proteasome signaling and contributes to various diseases, yet its mechanism in cardiac hypertrophy ...
Qiu-Yue Lin, Wei-Jia Yu, Jia-Xin Li, Wen-Xi Jiang, Shu-Jing Liu, Hai-Lian Bi, Hui-Hua Li   +6 more
doaj   +2 more sources

A rapid, accessible real-time PCR approach to identify UBA1 somatic mutations in VEXAS syndrome [PDF]

Frontiers in Medicine
BackgroundVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a severe adult-onset autoinflammatory disease caused by somatic mutations in the X-linked UBA1 gene, most commonly affecting codon 41.
Luisa Agnello, Luisa Agnello, Caterina Maria Gambino, Caterina Maria Gambino, Lidia La Barbera, Anna Masucci, Roberta Vassallo, Francesco Cacciabaudo, Mauro Midiri, Concetta Scazzone, Anna Maria Ciaccio, Giuliana Guggino, Marcello Ciaccio, Marcello Ciaccio   +13 more
doaj   +2 more sources

Cryo-EM structures of UBA6 reveal mechanisms of E1–E2 specificity and dual FAT10/ubiquitin thioester transfer [PDF]

Nature Communications
UBA1 and UBA6 define parallel ubiquitin (Ub) activation systems that perform non-overlapping roles in Ub and ubiquitin-like protein (Ubl) signaling. Whereas UBA1 supports the canonical Ub pathway, UBA6 also activates the Ubl FAT10, linking Ub signaling ...
Digant Nayak, Lijia Jia, Priscila dos Santos Bury, Eliza A. Ruben, Ankita Shukla, Anindita Nayak, Caleb M. Stratton, Pirouz Ebadi, Hee Cho, Anna A. Tumanova, Joyce T. Varughese, Lingmin Yuan, Fei Gao, Kristin E. Cano, Christopher Davies, Patrick Sung, Michaela U. Gack, Elizabeth V. Wasmuth, Shaun K. Olsen   +18 more
doaj   +2 more sources

Joint involvement in VEXAS and non-VEXAS clonal haematopoiesis: two clusters from a multicentre regional cohort [PDF]

RMD Open
Objective: To describe the joint manifestations associated with clonal haematopoiesis and to compare patients with and without VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome.
Olivier Vittecoq, Fabrice Jardin, Thierry Lequerre, Ygal Benhamou, Vincent Langlois, Baptiste de Maleprade, Gaetan Sauvetre, Pauline Brevet, Fabien Jastrzebski, Benjamin Membrey, Marine Avenel, Alexandre Curie, Guillaume Armengol, Aspasia Stamatoullas-Bastard   +13 more
doaj   +2 more sources

Case Report: Early-onset VEXAS syndrome with recurrent pulmonary inflammation and myelodysplasia: a diagnostic and therapeutic challenge [PDF]

Frontiers in Immunology
BackgroundVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly recognized adult-onset autoinflammatory disorder caused by somatic mutations in the UBA1 gene. It typically presents in older males with systemic inflammation,
Xianghong Jin, Xianyong Jiang, Yaping Liu, Miao Chen, Jiayuan Dai, Jin Xu, Min Shen   +6 more
doaj   +2 more sources

A fatal case of VEXAS syndrome with a brief review [PDF]

Journal of Vascular Surgery Cases and Innovative Techniques
VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a recently described adult-onset autoinflammatory condition characterized by somatic mutations in the X-linked UBA1 gene.
Grant Stoltman, BA, Rashid Skeik, BS, Jesse Manunga, MD, Nedaa Skeik, MD   +3 more
doaj   +2 more sources

Characterization of ubiquitin-activating enzyme Uba1 in the nucleus by its mammalian temperature-sensitive mutant. [PDF]

PLoS ONE, 2014
Temperature-sensitive (ts) CHO-K1 mutant tsTM3 exhibits chromosomal instability and cell-cycle arrest in the S to G2 phases with decreased DNA synthesis at the nonpermissive temperature, 39°C.
Kimihiko Sugaya, Kimihiko Sugaya, Yoshie Ishihara, Sonoe Inoue, Hideo Tsuji   +4 more
doaj   +1 more source

An adaptive stress response that confers cellular resilience to decreased ubiquitination

Nature Communications, 2023
Ubiquitination is a post-translational modification initiated by the E1 enzyme UBA1, which transfers ubiquitin to ~35 E2 ubiquitin-conjugating enzymes. While UBA1 loss is cell lethal, it remains unknown how partial reduction in UBA1 activity is endured ...
Liam C. Hunt, Vishwajeeth Pagala, Anna Stephan, Boer Xie, Kiran Kodali, Kanisha Kavdia, Yong-Dong Wang, Abbas Shirinifard, Michelle Curley, Flavia A. Graca, Yingxue Fu, Suresh Poudel, Yuxin Li, Xusheng Wang, Haiyan Tan, Junmin Peng, Fabio Demontis   +16 more
doaj   +1 more source