TSEN54 Antibody

Code CSB-PA008909
Size US$100
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Product Details

Uniprot No.
Target Names
TSEN54
Alternative Names
HsSen54 antibody; PCH2A antibody; PCH4 antibody; sen54 antibody; SEN54 homolog antibody; SEN54_HUMAN antibody; SEN54L antibody; tRNA-intron endonuclease SEN54 antibody; tRNA-splicing endonuclease subunit SEN54 antibody; Tsen54 antibody; TSEN54 tRNA splicing endonuclease subunit antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Synthesized peptide derived from the Internal region of Human TSEN54.
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form
Liquid
Tested Applications
WB, IHC, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:100-1:300
ELISA 1:20000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

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.
Gene References into Functions
  1. TSEN54 gene-related pontocerebellar hypoplasia type 2 presented with exaggerated startle response in cousins. PMID: 26701950
  2. A novel heterozygous mutation was found in the TSEN54 gene by c.254A > T(+) (p.E85V), which may be a new subtype of hereditary ataxia PMID: 24938831
  3. TSEN54 mutation causes a severe form of pontocerebellar hypoplasia type 1 in a family. PMID: 21468723
  4. The results demonistrated that not all cases of clinically defined pontocerebellar hypoplasia-4 result from mutations in TSEN54. PMID: 21383226
  5. We confirm that the common p.A307S mutation in TSEN54 is responsible for most of the patients with a PCH2 phenotype. PMID: 20956791
  6. In two subtypes, PCH2 and PCH4, we identified mutations in three of the four different subunits of the tRNA-splicing endonuclease complex. PMID: 18711368

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Involvement in disease
Pontocerebellar hypoplasia 4 (PCH4); Pontocerebellar hypoplasia 2A (PCH2A); Pontocerebellar hypoplasia 5 (PCH5)
Subcellular Location
Nucleus. Nucleus, nucleolus.
Protein Families
SEN54 family
Database Links

HGNC: 27561

OMIM: 225753

KEGG: hsa:283989

STRING: 9606.ENSP00000327487

UniGene: Hs.378501

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