SOBP (sine oculis binding protein homolog) is a nuclear zinc finger protein critical for craniofacial and inner ear development. SOBP functions as a co-factor that modulates the transcriptional activation of Six1 target genes, binding to and colocalizing with Six1 in the cell nucleus to regulate otic and craniofacial development 1. In mice, Sobp is essential for patterning the organ of Corti 2. SOBP is highly expressed in the brain limbic system during active synaptogenesis, a region regulating learning, memory, and affective behavior 2. Mutations in SOBP cause both syndromic and nonsyndromic intellectual disability (ID), with affected individuals displaying anterior maxillary protrusion, strabismus, temporal lobe epilepsy, and psychosis 2. SOBP variants are implicated in branchio-oto-renal syndrome, characterized by hearing loss and craniofacial/renal defects 1. Proteomics studies in Sobp-mutant mice revealed altered expression of 24 proteins, including dynamin and pacsin1, suggesting broader neurobiological effects 2. Beyond developmental roles, SOBP expression is dysregulated in non-Hodgkin B-cell lymphoma, where intragenic exon rearrangements occur 3. In ovarian carcinoma, SOBP downregulation correlates with inferior prognosis and enhanced immune cell infiltration, suggesting involvement in immune regulation 4. These findings indicate SOBP functions across developmental, neurological, and oncological contexts.