SKIC2 (SKI2 subunit of superkiller complex) encodes a DEVH-box RNA helicase that functions as a critical component of the SKI complex, which assists cytoplasmic mRNA surveillance and degradation pathways 1. The protein contains consensus sequences for RNA helicase structural motifs and exhibits ATPase activity 1. SKIC2 works in conjunction with SKIC3 (TTC37) to form the putative human SKI complex, a heterotetrameric cofactor of the cytoplasmic RNA exosome that ensures aberrant mRNA decay 2. The SKI complex plays essential roles in mRNA quality control by targeting mRNAs lacking proper stop codons through the non-stop decay pathway 2. Loss of SKIC2 function compromises the SKI mRNA surveillance pathway, as demonstrated in FOCAD deficiency studies where reduced SKIC2 levels led to altered mRNA degradation processes 3. Disease relevance is established through mutations in SKIC2 causing trichohepatoenteric syndrome type 2 (THES-2), a rare autosomal recessive disorder characterized by intractable neonatal diarrhea, growth restriction, facial dysmorphism, woolly hair, and immune disorders 24. Clinical management typically requires parenteral nutrition and immunoglobulin supplementation, with prognosis depending on complications from these interventions 25.