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

More general terms

• endonuclease
• enzyme catalytic subunit
• leukocyte receptor cluster member
• phosphoprotein

Component of

• tRNA-splicing endonuclease complex

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=79042
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:79042
• OMIM: https://mirror.omim.org/entry/608754
• OMIM: https://mirror.omim.org/entry/612390
• UniProt: http://www.uniprot.org/uniprot/Q9BSV6.html