TSEN54, Polyclonal Antibody

tRNA-splicing endonuclease subunit Sen54; tRNA-intron endonuclease Sen54; HsSen54; TSEN54; SEN54

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

The antibody detects endogenous levels of total TSEN54 protein.

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

1.0 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-TSEN54 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.Zody M.C., Nature 440:1045-1049(2006).Paushkin S.V., Cell 117:311-321(2004).Ota T., Nat. Genet. 36:40-45(2004).

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

Western blotting: 1:500~1:3000
Immunohistochemistry: 1:50~1:100

Western blot analysis of extracts from 3T3 cells and Jurkat cells, using TSEN54 antibody.

Immunohistochemistry analysis of paraffin-embedded human lung carcinoma tissue, using TSEN54 antibody.

19,981 Da

TSEN54 tRNA splicing endonuclease subunit

tRNA-splicing endonuclease subunit Sen54

tRNA-splicing endonuclease subunit Sen54

SEN54; HsSEN54  [Similar Products]

SEN54_HUMAN

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]

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.

Protein type: Nucleolus

Chromosomal Location of Human Ortholog: 17q25.1

Cellular Component: nucleolus

Biological Process: tRNA splicing; mRNA processing

Disease: Pontocerebellar Hypoplasia, Type 4; Pontocerebellar Hypoplasia, Type 2a

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