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tRNA-splicing endonuclease subunit Sen34

 
Known also as: Leukocyte receptor cluster member 5, tRNA-intron endonuclease Sen34, HsSen34

Known abbreviations: TSEN34, LENG5, SEN34

 

FUNCTION:
 
Constitutes one of the two 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. It probably carries the active site for 3'-splice site cleavage. 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. 
 
CATALYTIC ACTIVITY:
 
Endonucleolytic cleavage of pre-tRNA, producing 5'-hydroxy and 2',3'-cyclic phosphate termini, and specifically removing the intron.
 
SUBUNIT STRUCTURE:
 
tRNA splicing endonuclease is a heterotetramer composed of SEN2, SEN15, SEN34/LENG5 and SEN54. tRNA splicing endonuclease complex also contains proteins of the pre-mRNA 3'-end processing machinery such as CLP1, CPSF1, CPSF4 and CSTF2.
 
CELLULAR LOCALIZATION:
 
Nucleus. Nucleus › nucleolus. Note: May be transiently localized in the nucleolus. 
 
INVOLVEMENT IN DISEASE:
 
Defects in TSEN34 are the cause of pontocerebellar hypoplasia type 2C (PCH2C) [MIM:612390]. 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.



This protein can be a part of a given complexes: Activities in which tRNA-splicing endonuclease subunit Sen34 is involved: Pathways in which tRNA-splicing endonuclease subunit Sen34 is involved:

NCBI GI number(s): 116875842
260763974
116875840
260763975

Species: Homo sapiens

Links to other databases:

Database ID Link
Uniprot Q9BSV6 Q9BSV6
BRENDA - -
KEGG hsa:79042 hsa:79042
PFAM: PF01974
PF01974
InterPro: IPR011856
IPR006677
IPR006676
IPR016690
IPR011856
IPR006677
IPR006676
IPR016690
CATH: - -
SCOP: - -


Protein sequence:
MLVVEVANGRSLVWGAEAVQALRERLGVGGRTVGALPRGPRQNSRLGLPL
LLMPEEARLLAEIGAVTLVSAPRPDSRHHSLALTSFKRQQEESFQEQSAL
AAEARETRRQELLEKITEGQAAKKQKLEQASGASSSQEAGSSQAAKEDET
SDGQASGEQEEAGPSSSQAGPSNGVAPLPRSALLVQLATARPRPVKARPL
DWRVQSKDWPHAGRPAHELRYSIYRDLWERGFFLSAAGKFGGDFLVYPGD
PLRFHAHYIAQCWAPEDTIPLQDLVAAGRLGTSVRKTLLLCSPQPDGKVV
YTSLQWASLQ

TRNA-splicing endonuclease subunit Sen34 (Homo sapiens) is product of expression of TSEN34 gene.

References:

Title Authors Journal Publication date (Issue) PubMed ID
tRNA splicing endonuclease mutations cause pontocerebellar hypoplasia. Budde BS, Namavar Y, Barth PG, Poll-The BT, Nurnberg G, Becker C, van Ruissen F, Weterman MA, Fluiter K, te Beek ET, Aronica E, van der Knaap MS, Hohne W, Toliat MR, Crow YJ, Steinling M, Voit T, Roelenso F, Brussel W, Brockmann K, Kyllerman M, Boltshauser E, Hammersen G, Willemsen M, Basel-Vanagaite L, Krageloh-Mann I, de Vries LS, Sztriha L, Muntoni F, Ferrie CD, Battini R, Hennekam RC, Grillo E, Beemer FA, Stoets LM, Wollnik B, Nurnberg P, Baas F Nat Genet 2008-09-01 (40) 18711368
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, K Genome Res 2004-10-01 (14) 15489334
Identification of a human endonuclease complex reveals a link between tRNA splicing and pre-mRNA 3' end formation. Paushkin SV, Patel M, Furia BS, Peltz SW, Trotta CR Cell 2004-04-01 (117) 15109492
Complete sequencing and characterization of 21,243 full-length human cDNAs. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K Nat Genet 2004-02-01 (36) 14702039
The DNA sequence and biology of human chromosome 19. Grimwood J, Gordon LA, Olsen A, Terry A, Schmutz J, Lamerdin J, Hellsten U, Goodstein D, Couronne O, Tran-Gyamfi M Nature 2004-04-01 (428) 15057824
Extensive gene duplications and a large inversion characterize the human leukocyte receptor cluster. Wende H, Volz A, Ziegler A Immunogenetics 2000-07-01 (51) 10941842
Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach. Gauci S, Helbig AO, Slijper M, Krijgsveld J, Heck AJ, Mohammed S Anal Chem 2009-06-01 (81) 19413330
Initial characterization of the human central proteome. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J BMC Syst Biol 2011-01-01 (5) 21269460



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Last modification of this entry: Sept. 25, 2012.

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