SHQ1 is an essential chaperone protein that assembles H/ACA ribonucleoproteins (RNPs), including telomerase, by stabilizing and escorting the pseudouridine synthase dyskerin from the cytoplasm to the nucleus 1. In the nucleus, SHQ1 facilitates dyskerin's incorporation into H/ACA snoRNPs, which catalyze pseudouridine formation in ribosomal RNAs and promote pre-rRNA maturation 12. SHQ1 binds dyskerin through its conserved CS and Shq1-specific domains, protecting the enzyme from aggregation and non-specific RNA binding before H/ACA RNA assembly 32. Loss-of-function mutations in SHQ1 impair dyskerin stabilization and H/ACA snoRNP biogenesis, leading to accumulated unprocessed pre-rRNAs and reduced ribosome production 1. Clinically, biallelic SHQ1 variants cause neurodevelopmental disorders with early-onset hypotonia, paroxysmal dystonia, and cerebellar degeneration, associated with dopaminergic pathway dysfunction 4. Additionally, SHQ1 deletion at chromosome 3-14 acts as a tumor suppressor, cooperating with PTEN loss to accelerate prostate cancer progression 56, while SHQ1 promotes chemotherapy sensitivity in hepatocellular carcinoma through ER-stress response mechanisms 7.