Anti-TSEN54 Antibody (CAB15227)

Antibody Name:	Anti-TSEN54 Antibody
Antibody SKU:	CAB15227
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human TSEN54 (NP_997229.2).

Application:	WB
Recommended Dilution:	WB 1:500 - 1:2000
Reactivity:	Human, Mouse, Rat
Positive Samples:	HeLa, MCF7, 293T, Mouse kidney, Rat kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human TSEN54 (NP_997229.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MEPE PEPA AVEV PAGR VLSA RELF AARS RSQK LPQR SHGP KDFL PDGS AAQA ERLR RCRE ELWQ LLAE QRVE RLGS LVAA EWRP EEGF VELK SPAG KFWQ TMGF SEQG RQRL HPEE ALYL LECG SIHL FHQD LPLS IQEA YQLL LTDH TVTF LQYQ VFSH LKRL GYVV RRFQ PSSV LSPY ERQL NLDA SVQH LEDG DGKR KRSS SSPR SINK KAKA LDNS LQPK SLAA SSPP PCSQ PSQC PEEK PQES SPMK GPGG PFQL LGSL GPSP GPAR EGVG CSWE SGRA ENGV TGAG KRRW NFEQ
Gene ID:	283989
Uniprot:	Q7Z6J9
Cellular Location:	Nucleus, nucleolus
Calculated MW:	19kDa/58kDa
Observed MW:	59kDa

Synonyms:	TSEN54, PCH2A, PCH4, PCH5, SEN54L, sen54
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.

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

Western blot analysis of extracts of various cell lines, using TSEN54 Rabbit pAb (CAB15227) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.

Antibodies

TSEN54 Antibody (PACO03926)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)