Superkiller complex protein 3 (SKI3)

Superkiller complex protein 3 (SKI3) is a core structural subunit of the cytoplasmic SKI complex, which partners with the RNA exosome to facilitate the 3'-to-5' degradation of cytoplasmic mRNAs as part of mRNA surveillance and decay pathways[1][2][3][4][5]. SKI3 is a large tetratricopeptide repeat (TPR) protein, acting as a scaffold within the SKI complex that assembles with the RNA helicase SKI2 and the WD repeat protein SKI8 in a conserved 1:1:2 stoichiometry[1][2][3][5]. While SKI2 provides RNA helicase activity, SKI3 and SKI8 are considered non-enzymatic structural components that coordinate complex assembly and modulation[1][2][3]. The SKI complex is conserved from yeast (where it was historically associated with viral resistance) to humans, where the SKI3 homolog is encoded by the TTC37 gene[1][2][3]. In humans, biallelic mutations in TTC37/SKI3 cause tricho-hepatic-enteric syndrome, a rare multisystem disorder. In general, SKI3 is not considered a direct therapeutic target (such as a receptor, enzyme, or transporter), nor are there currently drugs known to act on SKI3 or the SKI complex in clinical use.

Other names

SKIC3KIAA0372TTC37ThespinTHESTPR repeat protein 37Tetratricopeptide repeat protein 37Tricho-hepatic-enteric syndrome proteinSki3p (yeast)Superkiller complex protein 3 homologSki complex component SKI3