| ELK.No | ES4223 |
| Product name | UBA1 rabbit pAb |
| Reactivity | Human;Mouse;Rat |
| Applications | WB;ELISA |
| Other name | UBA1; A1S9T; UBE1; Ubiquitin-like modifier-activating enzyme 1; Protein A1S9; Ubiquitin-activating enzyme E1 |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 7317 |
| Human Swiss-Prot | P22314 |
| Source | Rabbit |
| Isotype | IgG |
| Target | UBA1 |
| Fields | >>Ubiquitin mediated proteolysis;>>Parkinson disease;>>Pathways of neurodegeneration - multiple diseases |
| Gene name | UBA1 |
| Protein name | Ubiquitin-like modifier-activating enzyme 1 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 22201 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q02053 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 314432 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q5U300 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | The antiserum was produced against synthesized peptide derived from the N-terminal region of human UBA1. AA range:91-140 |
| Specificity | UBA1 Polyclonal Antibody detects endogenous levels of UBA1 protein. |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/20000. Not yet tested in other applications. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 118kD |
| 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], |
| Function | 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 |
| Subcellular location | Cytoplasm . Mitochondrion . Nucleus .; [Isoform 1]: Nucleus .; [Isoform 2]: Cytoplasm . |
| Expression | Detected in erythrocytes (at protein level). Ubiquitous. |

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