UBE1, active

Background Information:

UBE1 (also known as UBA1) catalyzes the initiating step of the ubiquitin conjugation cascade, a process that targets intracellular proteins for degradation via the ubiquitin–proteasome system. The enzyme activates ubiquitin through an ATP-dependent adenylation of its C-terminal glycine residue, followed by the formation of a high-energy thioester bond between this glycine and a conserved active-site cysteine within UBE1, generating a ubiquitin–E1 thioester intermediate and releasing AMP. UBE1 is critical for the assembly of radiation-induced nuclear foci, efficient DNA double-strand break repair, and the cellular response to replication stress. It facilitates the recruitment of key DNA damage response mediators, including TP53BP1 and BRCA1, to sites of DNA lesions. Aberrant UBE1 activity or expression has been implicated in the pathogenesis of various cancers, as well as inflammatory and neurodegenerative disorders.

Target Class:

Ubiquitination-related

Family:

ubiquitin-activating E1

Sub Family:

Ligase

Protein Name:

UBE1

Protein Aliases:

UBA1

Accession Number:

NM_003334.3

UniProt Number:

P22314

Gene Name:

UBA1

Gene ID:

7317

Gene Aliases:

A1S9; A1ST; GXP1; UBE1; A1S9T; AMCX1; POC20; SMAX2; UBA1A; UBE1X; VEXAS; CFAP124

Target Species:

Human