GEMIN5 is an RNA-binding protein essential for spliceosomal snRNP assembly and translation regulation. Within the SMN complex, GEMIN5 recognizes and delivers small nuclear RNAs (snRNAs) to facilitate core snRNP formation, the building blocks of spliceosomes required for pre-mRNA splicing 1. GEMIN5 recognizes snRNAs through its N-terminal WD repeat domains and binds the 7-methylguanosine cap of RNA molecules 2. Beyond snRNP biogenesis, GEMIN5 regulates translation through multiple mechanisms: it binds the 3'-UTR of SMN1 mRNA to regulate its translation 2, modulates polysome partitioning via ribosome interaction 2, and acts as an m6A-dependent translator of select mRNAs like FZR1 3. Biallelic GEMIN5 mutations cause a neurodevelopmental disorder characterized by developmental delay, hypotonia, and cerebellar ataxia through disrupted snRNP assembly and altered mRNA splicing patterns 1. GEMIN5 dysfunction also impairs mitochondrial function via CoQ10 biosynthesis deficiency, a defect potentially addressable through CoQ10 supplementation 4. SMN acts as a genetic modifier of GEMIN5-related neurodegeneration by regulating GEMIN5 expression via Tudor domain interaction 5.