Ubiquitin-like modifier-activating enzyme 1

Definition:

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.

References:

[1]. J C Cook, et al. Isoforms of mammalian ubiquitin-activating enzyme. J Biol Chem. 1992 Dec 5;267(34):24315-21. [Content Brief]

[2]. David B Beck, et al. Somatic Mutations in UBA1 and Severe Adult-Onset Autoinflammatory Disease. N Engl J Med. 2020 Dec 31;383(27):2628-2638. [Content Brief]

[3]. Pavel Moudry, et al. Ubiquitin-activating enzyme UBA1 is required for cellular response to DNA damage. Cell Cycle. 2012 Apr 15;11(8):1573-82. [Content Brief]

[4]. D Ayusawa, et al. Complementation by a cloned human ubiquitin-activating enzyme E1 of the S-phase-arrested mouse FM3A cell mutant with thermolabile E1. Cell Struct Funct. 1992 Apr;17(2):113-22. [Content Brief]

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