Price range: $268.00 through $328.00
Background:
This gene encodes a subunit of the tRNA splicing endonuclease complex, which catalyzes the removal of introns from precursor tRNAs. The complex is also implicated in pre-mRNA 3-prime end processing. Mutations in this gene result in pontocerebellar hypoplasia type 2.[provided by RefSeq, Oct 2009],
| Size | 50μL/100μL |
| Target | TSEN54 |
| Reactivity | Human, Mouse |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | |
| MW(Observed) | 60kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; IHC 1:100-1:300; ELISA 1:20000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| 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 human TSEN54. AA range:261-310 |
| Specificity | TSEN54 Polyclonal Antibody detects endogenous levels of TSEN54 protein. |
| Gene Name | TSEN54 |
| Protein Name | tRNA-splicing endonuclease subunit Sen54 |
| Other Name | TSEN54; / SEN54; / tRNA-splicing endonuclease subunit Sen54; / SEN54 homolog; / HsSEN54; / tRNA-intron endonuclease Sen54 |
| Species | Human |
| Gene ID | 283989 |
| Uniprot | Q7Z6J9, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q8C2A2 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Nucleus . Nucleus, nucleolus . May be transiently localized in the nucleolus. . |
| Tissue Expression | Blood,Cerebellum,Uterus, |
| Signaling_pathway | |
| Research Areas | |
| Function | Disease:Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 2A (PCH2A) [MIM:277470]. PCH type 2 is characterized by progressive microcephaly from birth combined with extrapyramidal dyskinesia and chorea, epilepsy, and normal spinal cord findings.,Disease:Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 4 (PCH4) [MIM:225753]. Pontocerebellar hypoplasia (PCH) is a heterogeneous group of disorders characterized by an abnormally small cerebellum and brainstem. PCH4 is characterized by severe course and early lethality.,Function:Non-catalytic subunit of the tRNA-splicing endonuclease complex, a complex responsible for identification and cleavage of the splice sites in pre-tRNA. It cleaves pre-tRNA at the 5' and 3' splice sites to release the intron. The products are an intron and two tRNA half-molecules bearing 2',3' cyclic phosphate and 5'-OH termini. There are no conserved sequences at the splice sites, but the intron is invariably located at the same site in the gene, placing the splice sites an invariant distance from the constant structural features of the tRNA body. The tRNA splicing endonuclease is also involved in mRNA processing via its association with pre-mRNA 3' end processing factors, establishing a link between pre-tRNA splicing and pre-mRNA 3' end formation, suggesting that the endonuclease subunits function in multiple RNA-processing events.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,similarity:Belongs to the SEN54 family.,subcellular location:May be transiently localized in the nucleolus.,subunit:tRNA splicing endonuclease is a heterotetramer composed of SEN2, SEN15, SEN34/LENG5 and SEN54. tRNA splicing endonuclease complex also contains proteins of the Pre-mRNA 3' end processing machinery such as CLP1, CPSF1, CPSF4 and CSTF2. Also belongs to a complex containing isoform 2 of SEN2., |
