Target：TSEN54

Gene Name：TSEN54

Protein Name：tRNA-splicing endonuclease subunit Sen54

Human Gene Id：283989

Human Swiss Prot No：Q7Z6J9

Mouse Swiss Prot No：Q8C2A2

Immunogen：The antiserum was produced against synthesized peptide derived from human TSEN54. AA range:261-310

Specificity：TSEN54 Polyclonal Antibody detects endogenous levels of TSEN54 protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200

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

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：TSEN54;SEN54;tRNA-splicing endonuclease subunit Sen54;SEN54 homolog;HsSEN54;tRNA-intron endonuclease Sen54

Observed Band(KD)：60kD

Background： 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],

Function：disease:Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 2A (PCH2A) [MIM:277470]. PCH type 2 is characterized by progressive microcephaly from birth combined with extrapyramidal dyskinesia and chorea, epilepsy, and normal spinal cord findings.,disease:Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 4 (PCH4) [MIM:225753]. 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.,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 a

Subcellular Location：Nucleus . Nucleus, nucleolus . May be transiently localized in the nucleolus. .

Expression： Blood,Cerebellum,Uterus,