tRNA splicing endonuclease subunit 34 (TSEN34)

TSEN34 is a catalytic subunit of the tRNA splicing endonuclease complex, required for accurate excision of introns from precursor tRNA molecules in eukaryotic cells. It cleaves at the 3′ splice site and also interacts with other complex subunits (notably TSEN2, TSEN15, and TSEN54) to facilitate tRNA maturation and efficient protein translation. TSEN34's function extends to mRNA 3′-end processing through association with additional factors, thus linking tRNA and mRNA processing pathways. Mutations in TSEN34 result in pontocerebellar hypoplasia (PCH) type 2C, leading to developmental delay, neurological deficits, and intellectual disability, demonstrating its essential role in brain development and cellular homeostasis. No drugs are currently known to directly target TSEN34, and any potential therapeutic modulation is limited by its critical cellular functions and associated safety risks.

Other names

SEN34LENG5SEN34LHsSen34Leukocyte receptor cluster member 5tRNA-intron endonuclease Sen34PCH2CCTD-3093M3.1

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