Human tRNA splicing endonuclease 54 homolog (S. cerevisiae) ELISA Kit | TSEN54 elisa kit
Human tRNA-splicing endonuclease subunit Sen54, TSEN54 ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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 Description
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
Research Articles on TSEN54
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Product Notes
The Human TSEN54 tsen54 (Catalog #AAA9333179) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9333179 ELISA Kit recognizes Human TSEN54. It is sometimes possible for the material contained within the vial of "tRNA splicing endonuclease 54 homolog (S. cerevisiae), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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