DKC1 encodes dyskerin, a pseudouridine synthase essential for telomere maintenance and ribosomal RNA modification. The protein functions as a core component of the telomerase holoenzyme complex and catalyzes pseudouridylation of ribosomal and small nuclear RNAs through its box H/ACA snoRNP interactions 1. DKC1 also stabilizes mRNAs encoding ribosomal proteins, thereby promoting cell proliferation 2. Pathogenic DKC1 mutations cause X-linked dyskeratosis congenita, a telomere disease characterized by bone marrow failure, ectodermal dysplasia, and cancer predisposition. Affected individuals exhibit critically short telomeres and reduced pseudouridine stoichiometry in rRNA 34. Female carriers of DKC1 mutations show skewed X-chromosome X in hematopoietic and fibroblast cells, reflecting selection against DKC1-deficient cells 5. Beyond inherited disease, DKC1 is significantly upregulated in colorectal cancer tissues and associated with poorer overall and disease-free survival across multiple cancer types 26. The pyrazofurin–trametinib combination synergistically restrains colorectal cancer growth in cell and xenograft models 2, identifying DKC1 inhibition combined with MEK inhibition as a candidate therapeutic strategy.