UBA1 Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss

bsm-63004R

重组兔单抗

UBA1 Recombinant Rabbit mAb

泛素激活酶E1重组兔单抗

A1S9; A1S9T; A1ST; AMCX1; CFAP124; GXP1; POC20; SMAX2; UBA1A; UBE1; UBE1X; VEXAS; Sbx; Ube-1; UBA1_HUMAN; UBA1; Protein A1S9; Ubiquitin-activating enzyme E1; 6.2.1.45; UBA1_MOUSE; Ubiquitin-activating enzyme E1 X; Ubiquitin-like modifier-activating enzyme 1 X; Ube1ax; UBA1_RAT; ubiquitin like modifier activating enzyme 1; ubiquitin-activating enzyme E1 (A1S9T and BN75 temperature sensitivity complementing); ubiquitin-like modifier activating enzyme 1; UBA1, ubiquitin-activating enzyme E1 homolog (yeast); POC20 centriolar protein homolog (Chlamydomonas)

Rabbit

A synthesized peptide derived from human UBA1: 200-255/1058

IgG

Liquid

affinity purified by Protein A

Recombinant

7D16

118 kDa

115-120 kDa

10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.

Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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 an ubiquitin-E1 thioester and free AMP.

UBA1

Ubiquitin-like modifier-activating enzyme 1

Monomer (By similarity). Interacts with GAN (via BTB domain).

ISGylated.

Defects in UBA1 are the cause of spinal muscular atrophy X-linked type 2 (SMAX2) [MIM:301830]; 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.

Belongs to the ubiquitin-activating E1 family.

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 an ubiquitin-E1 thioester and free AMP.