9mc4 | pdb_00009mc4
From Proteopedia
Cryo-EM structure of Human UBA1-UBE2O-Ub -Transthiolation state 4
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Structural highlights
DiseaseUBA1_HUMAN X-linked distal arthrogryposis multiplex congenita. The disease is caused by mutations affecting the gene represented in this entry. FunctionUBA1_HUMAN Catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation through the ubiquitin-proteasome system. Activates ubiquitin by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding a ubiquitin-E1 thioester and free AMP. Essential for the formation of radiation-induced foci, timely DNA repair and for response to replication stress. Promotes the recruitment of TP53BP1 and BRCA1 at DNA damage sites.[1] References
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This page was last modified 07:47, 25 March 2026.