Price range: $268.00 through $328.00
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],
| Size | 50μL/100μL |
| Target | UBA1 |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, ELISA |
| MW(Calculated) | |
| MW(Observed) | 118kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; ELISA 1:20000; Not yet tested in other applications. |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| 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. |
| Gene Name | UBA1 |
| Protein Name | Ubiquitin-like modifier-activating enzyme 1 |
| Other Name | UBA1; / A1S9T; / UBE1; / Ubiquitin-like modifier-activating enzyme 1; / Protein A1S9; / Ubiquitin-activating enzyme E1 |
| Species | Human |
| Gene ID | 7317 |
| Uniprot | P22314, |
| Species.1 | Mouse |
| Gene ID.1 | 22201 |
| Uniprot.1 | Q02053, |
| Species.2 | Rat |
| Gene ID.2 | 314432 |
| Uniprot.2 | Q5U300 |
| Cellular Localization | Cytoplasm . Mitochondrion . Nucleus .; [Isoform 1]: Nucleus .; [Isoform 2]: Cytoplasm . |
| Tissue Expression | Detected in erythrocytes (at protein level). Ubiquitous. |
| Signaling_pathway | Human Diseases >> Neurodegenerative disease >> Parkinson disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases;Genetic Information Processing >> Folding, sorting and degradation >> Ubiquitin mediated proteolysis |
| Research Areas | >>Ubiquitin mediated proteolysis; / >>Parkinson disease; / >>Pathways of neurodegeneration - multiple diseases |
| 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 moieties at a time, with a new ubiquitin forming an adenylate intermediate as the previous one is transferred to the thiol site.,pathway:Protein modification; protein ubiquitination.,similarity:Belongs to the ubiquitin-activating E1 family.,subunit:Monomer (By similarity). Interacts with GAN (via BTB domain)., |
