• 靶点：
• UBA1

• 简介：
• >>Ubiquitin mediated proteolysis;>>Parkinson disease;>>Pathways of neurodegeneration - multiple diseases

• 基因名称：
• UBA1

• 蛋白名称：
• Ubiquitin-like modifier-activating enzyme 1

• Human Gene Id：
• 7317

• Human Swiss Prot No：
• P22314

• Mouse Gene Id：
• 22201

• Mouse Swiss Prot No：
• Q02053

• Rat Gene Id：
• 314432

• Rat Swiss Prot No：
• Q5U300

• 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.

• 组成：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1:500 - 1:2000. ELISA: 1:20000. Not yet tested in other applications.

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 其他名称：
• UBA1;A1S9T;UBE1;Ubiquitin-like modifier-activating enzyme 1;Protein A1S9;Ubiquitin-activating enzyme E1

• 实测条带：
• 118kD

• 背景：
• 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

• 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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