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tRNA-splicing endonuclease subunit sen34; leukocyte receptor cluster member 5; tRNA-intron endonuclease sen34; Hssen34 (TSEN34, LENG5, SEN34)
Function:
- constitutes one of the two catalytic subunit of the tRNA-splicing endonuclease complex
- probably carries the active site for 3'-splice site cleavage
Structure:
- belongs to the tRNA-intron endonuclease family
Compartment:
- nucleus, nucleolus
- may be transiently localized in the nucleolus
Pathology:
- defects in TSEN34 are the cause of pontocerebellar hypoplasia type 2C
Genetics:
- gene belongs to the leukocyte receptor cluster (LRC) present on 19q13.4
General
endonuclease
enzyme catalytic subunit
leukocyte receptor cluster member
phosphoprotein
Properties
SIZE: entity length = 310 aa
MW = 34 kD
COMPARTMENT: cell nucleus
MOTIF: Ser phosphorylation site {S134}
Ser phosphorylation site {S135}
Ser phosphorylation site {S136}
tyrosine residue {Y247}
histidine residue {H255}
lysine residue {K286}
Database Correlations
OMIM correlations
UniProt Q9BSV6
Pfam PF01974
Entrez Gene 79042
Kegg hsa:79042
ENZYME 3.1.27.9
References
UniProt :accession Q9BSV6
Component-of
tRNA-splicing endonuclease complex