Description
| 抗体名: | TSEN2 Antibody (PACO44008) |
| 抗体コード: | PACO44008 |
| サイズ: | 50ul |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB, IHC |
| 推奨される希釈: | ELISA:1:2000-1:10000, WB:1:200-1:1000, IHC:1:50-1:200 |
| 反応性: | Human |
| 免疫原: | Recombinant Human tRNA-splicing endonuclease subunit Sen2 protein (1-220AA) |
| 憲法: | Liquid |
| ストレージバッファ: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| 精製方法: | Antigen Affinity Purified |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
 | Western blot. All lanes: TSEN2 antibody at 5µg/ml + 293T whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 54, 52, 50, 48, 46 kDa. Observed band size: 54 kDa.. |
 | Immunohistochemistry of paraffin-embedded human brain tissue using PACO44008 at dilution of 1:100. |
| バックグラウンド: | Constitutes one of the two 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. Isoform 1 probably carries the active site for 5'-splice site cleavage. 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. Isoform 2 is responsible for processing a yet unknown RNA substrate. The complex containing isoform 2 is not able to cleave pre-tRNAs properly, although it retains endonucleolytic activity. |
| シノニム: | tRNA-splicing endonuclease subunit Sen2 (EC 4.6.1.16) (tRNA-intron endonuclease Sen2) (HsSen2), TSEN2, SEN2 |
| UniProt Protein Function: | SEN2: Constitutes one of the two 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. Isoform 1 probably carries the active site for 5'-splice site cleavage. 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. Isoform 2 is responsible for processing a yet unknown RNA substrate. The complex containing isoform 2 is not able to cleave pre-tRNAs properly, although it retains endonucleolytic activity. Defects in TSEN2 are the cause of pontocerebellar hypoplasia type 2B (PCH2B). Pontocerebellar hypoplasia (PCH) is a heterogeneous group of disorders characterized by an abnormally small cerebellum and brainstem. PCH type 2 is characterized by progressive microcephaly from birth combined with extrapyramidal dyskinesia and chorea, epilepsy, and normal spinal cord findings. Belongs to the tRNA-intron endonuclease family. 3 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Ribonuclease; EC 4.6.1.16; RNA processing; Nucleolus Chromosomal Location of Human Ortholog: 3p25.2 Cellular Component: centrosome; cytoplasm; nucleoplasm; tRNA-intron endonuclease complex Molecular Function:protein binding; tRNA-intron endonuclease activity Biological Process: tRNA splicing; tRNA-type intron splice site recognition and cleavage Disease: Pontocerebellar Hypoplasia, Type 2b |
| NCBI Summary: | This gene encodes one of the subunits of the tRNA splicing endonuclease. This endonuclease catalyzes the first step in RNA splicing which is the removal of introns. Mutations in this gene have been associated with pontocerebellar hypoplasia type 2. A pseudogene has been identified on chromosome 4. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Feb 2009] |
| UniProt Code: | Q8NCE0 |
| NCBI GenInfo Identifier: | 50428914 |
| NCBI Gene ID: | 80746 |
| NCBI Accession: | Q8NCE0.2 |
| UniProt Secondary Accession: | Q8NCE0,Q8WTW7, Q9BPU7, B7Z6K1, C9IZI7, G5E9Q3, |
| UniProt Related Accession: | Q8NCE0 |
| Molecular Weight: | 45,532 Da |
| NCBI Full Name: | tRNA-splicing endonuclease subunit Sen2 |
| NCBI Synonym Full Names: | tRNA splicing endonuclease subunit 2 |
| NCBI Official Symbol: | TSEN2 |
| NCBI Official Synonym Symbols: | SEN2; PCH2B; SEN2L |
| NCBI Protein Information: | tRNA-splicing endonuclease subunit Sen2 |
| UniProt Protein Name: | tRNA-splicing endonuclease subunit Sen2 |
| UniProt Synonym Protein Names: | tRNA-intron endonuclease Sen2; HsSen2 |
| UniProt Gene Name: | TSEN2 |
| UniProt Entry Name: | SEN2_HUMAN |
