免疫原
The antiserum was produced against synthesized peptide derived from the N-terminal region of human UBA1. AA range:91-140
特异性
UBA1 Polyclonal Antibody detects endogenous levels of UBA1 protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/20000. Not yet tested in other applications.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（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. [provided by RefSeq, Jul 2008],
功能
disease: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.,function: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.,miscellaneous:There are two active sites within the E1 molecule, allowing it to accommodate two ubiquitin mo
蛋白名称
Ubiquitin-like modifier-activating enzyme 1
其他名称
UBA1; A1S9T; UBE1; Ubiquitin-like modifier-activating enzyme 1; Protein A1S9; Ubiquitin-activating enzyme E1
Fields
>>Ubiquitin mediated proteolysis;>>Parkinson disease;>>Pathways of neurodegeneration - multiple diseases
细胞定位
Cytoplasm . Mitochondrion . Nucleus .; [Isoform 1]: Nucleus .; [Isoform 2]: Cytoplasm .
组织表达
Detected in erythrocytes (at protein level). Ubiquitous.
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