TSEN54 Antibody (PACO03926)
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주문- SKU:
- PACO03926
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | TSEN54 Antibody |
Antibody SKU: | PACO03926 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | WB:1:500-1:2000, IHC:1:100-1:300 |
Species Reactivity: | Human |
Immunogen: | synthesized peptide derived from the Internal region of human TSEN54. |
Form: | Liquid |
Storage Buffer: | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Purification Method: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Synonyms: | TSEN54; SEN54; tRNA-splicing endonuclease subunit Sen54; SEN54 homolog; HsSEN54; tRNA-intron endonuclease Sen54 |
UniProt Protein Function: | TSEN54: 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. Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 4 (PCH4). 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. Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 2A (PCH2A). 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 SEN54 family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Nucleolus Chromosomal Location of Human Ortholog: 17q25.1 Cellular Component: nucleolus; nucleoplasm; tRNA-intron endonuclease complex Molecular Function:protein binding; tRNA-intron endonuclease activity Biological Process: gene expression; mRNA processing; tRNA processing; tRNA splicing; tRNA-type intron splice site recognition and cleavage Disease: Pontocerebellar Hypoplasia, Type 2a; Pontocerebellar Hypoplasia, Type 4 |
NCBI Summary: | 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] |
UniProt Code: | Q7Z6J9 |
NCBI GenInfo Identifier: | 296452961 |
NCBI Gene ID: | 283989 |
NCBI Accession: | Q7Z6J9.3 |
UniProt Secondary Accession: | Q7Z6J9,Q86WV3, Q86XE4, Q8N9H2, |
UniProt Related Accession: | Q7Z6J9 |
Molecular Weight: | 19,981 Da |
NCBI Full Name: | tRNA-splicing endonuclease subunit Sen54 |
NCBI Synonym Full Names: | tRNA splicing endonuclease subunit 54 |
NCBI Official Symbol: | TSEN54 |
NCBI Official Synonym Symbols: | PCH4; PCH5; PCH2A; sen54; SEN54L |
NCBI Protein Information: | tRNA-splicing endonuclease subunit Sen54 |
UniProt Protein Name: | tRNA-splicing endonuclease subunit Sen54 |
UniProt Synonym Protein Names: | SEN54 homolog; HsSEN54; tRNA-intron endonuclease Sen54 |
Protein Family: | tRNA-splicing endonuclease |
UniProt Gene Name: | TSEN54 |
UniProt Entry Name: | SEN54_HUMAN |