Recombinant Human Ubiquitin-like modifier-activating enzyme 1 (UBA1), Truncated

Uniprot Id

P22314

Target Species

Human

Target Name

UBA1

Target Full Name

Ubiquitin-like modifier-activating enzyme 1

Target Function

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.

Target Involvement

Spinal muscular atrophy X-linked 2 (SMAX2)

Target Subcellular Location

Cytoplasm. Mitochondrion. Nucleus.; [Isoform 1]: Nucleus.; [Isoform 2]: Cytoplasm.

Target Protein Families

Ubiquitin-activating E1 family

Target Tissue Specificity

Detected in erythrocytes (at protein level). Ubiquitous.

Target Synonyms

A1S9; A1S9 protein; A1S9T and BN75 temperature sensitivity complementing; A1S9T; A1ST; AMCX1; CFAP124; CTD-2522E6.1; GXP 1; GXP1; MGC4781; POC20; POC20 centriolar protein homolog; Protein A1S9; SMAX2; Uba1; UBA1, ubiquitin-activating enzyme E1 homolog A; UBA1_HUMAN; UBA1A; UBE 1; UBE 1X; UBE1; UBE1X; Ubiquitin activating enzyme E1; Ubiquitin-activating enzyme E1; Ubiquitin-like modifier-activating enzyme 1

Target Background

The protein encoded by this gene catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. This gene complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair. It is part of a gene cluster on chromosome Xp11.23. Alternatively spliced transcript variants that encode the same protein have been described.