SMARCC1 (SWI/SNF related BAF chr3 remodeling complex subunit C1) is a core component of chr3 remodeling complexes that regulates gene transcription through ATP-dependent alteration of DNA-histone contacts 12. SMARCC1 belongs to both neural progenitor-specific (npBAF) and neuron-specific (nBAF) chr3 remodeling complexes, which undergo compositional switching during neural development to regulate neural stem cell self-renewal and neuronal differentiation [UniProt annotations]. Mechanistically, SMARCC1 can be post-translationally modified through succination following DNA damage, which destabilizes the chr3-remodeling complex to modulate p53-regulated cell cycle genes 3. Additionally, SMARCC1 recruits the SWI/SNF complex to chr3 to drive transcription of target genes such as IGF2BP2 in cancer cells 4. Clinically, de novo and inherited loss-of-function variants in SMARCC1 cause congenital hydrocephalus with aqueductal stenosis, developmental delay, and structural brain/cardiac defects—a condition termed 'SMARCC1-associated developmental dysgenesis syndrome' 5. SMARCC1 variants account for a significant proportion of sporadic congenital hydrocephalus cases and demonstrate autosomal dominant inheritance with incomplete penetrance 67. Functional studies in Xenopus show that SMARCC1 depletion impairs neural progenitor gene expression, including transcription factors essential for midgestational neurogenesis, supporting a neural stem cell paradigm of disease pathogenesis 58.