E1 Ubiquitin Activating Enzyme / UBA1 (Protein | Antibody | cDNA Clone | ELISA Kit)

All E1 Ubiquitin Activating Enzyme / UBA1 reagents are produced in house and quality controlled, including 1 E1 Ubiquitin Activating Enzyme / UBA1 Antibody, 38 E1 Ubiquitin Activating Enzyme / UBA1 Gene, 1 E1 Ubiquitin Activating Enzyme / UBA1 IP Kit, 1 E1 Ubiquitin Activating Enzyme / UBA1 Lysate, 1 E1 Ubiquitin Activating Enzyme / UBA1 Protein, 1 E1 Ubiquitin Activating Enzyme / UBA1 qPCR. All E1 Ubiquitin Activating Enzyme / UBA1 reagents are ready to use.

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E1 Ubiquitin Activating Enzyme / UBA1 Background

UBE1, also known as UBA1, belongs to the ubiquitin-activating E1 family. UBE1 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. UBE1 catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. It also catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation 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 an ubiquitin-E1 thioester and free AMP. Defects in UBA1 can cause spinal muscular atrophy X-linked type 2 (SMAX2), also known as X-linked lethal infantile spinal muscular atrophy, distal X-linked arthrogryposis multiplex congenita or X-linked arthrogryposis type 1 (AMCX1). Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. SMAX2 is a lethal infantile form presenting with hypotonia, areflexia, and multiple congenital contractures.

E1 Ubiquitin Activating Enzyme / UBA1 References

  • Jin J, et al. (2007) Dual E1 activation systems for ubiquitin differentially regulate E2 enzyme charging. Nature. 447(7148):1135-8.
  • Xia T, et al. (2007) Chaperone-dependent E3 ligase CHIP ubiquitinates and mediates proteasomal degradation of soluble guanylyl cyclase. Am J Physiol Heart Circ Physiol. 293(5):H3080-7.
  • Pridgeon JW, et al. (2009) Proteomic analysis reveals Hrs UIM-mediated ubiquitin signaling in multiple cellular processes. FEBS J. 276(1):118-31.