SCNM1 (sodium channel modifier 1) is a zinc finger protein that functions as a critical component of the human minor spliceosome, a specialized splicing machinery that processes the rare U12-type introns found in approximately 0.5% of human genes 1. The protein structurally mimics the SF3a complex of the major spliceosome and stabilizes the catalytic center of the minor spliceosome 1. SCNM1 demonstrates direct splicing activity through interactions with spliceosomal proteins including U1-70K and core Sm proteins, and functionally facilitates recognition of non-consensus splice donor sites 2. Biallelic loss-of-function SCNM1 variants cause orofaciodigital syndrome (OFD), a ciliopathy characterized by craniofacial, oral, and digital abnormalities, alongside neurodevelopmental disorders and oculomotor apraxia 34. SCNM1 deficiency impairs splicing of U12-intron-containing genes encoding ciliary and basal body proteins (TMEM107, FAM92A, CIBAR1), resulting in abnormally elongated primary cilia and defective Hedgehog signaling 34. In hepatocellular carcinoma (HCC), SCNM1 is frequently amplified on chromosome 1 and its overexpression promotes tumor growth, suppresses apoptosis, and regulates protein degradation pathways via DERL2 and BAG6, identifying it as a cancer-specific therapeutic target 5.