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CCHC-type zinc finger nucleic acid binding protein (CNBP) is a conserved nucleic acid-binding protein with seven CCHC-type zinc finger domains, preferentially binding single-stranded DNA and RNA. CNBP regulates gene expression at both the transcriptional and translational levels, in part by interacting with and remodeling G-rich sequences and G-quadruplex structures, and is essential for normal embryonic, neural, and muscle development. Pathogenic expansion of a CCTG repeat in the CNBP gene is the genetic basis of myotonic dystrophy type 2 (DM2), a disorder characterized by progressive muscle weakness. CNBP also contributes to tumor biology by modulating oncogenic transcriptional programs and translation of growth-related mRNAs. Its wide-reaching functions and pivotal biological roles make it a plausible—but challenging—therapeutic target, and its CCTG expansion serves as a validated biomarker for DM2 diagnosis.
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