TSPOAP1 (TSPO associated protein 1) encodes RIMBP1 (RIM-binding protein 1), a presynaptic active-zone protein essential for neurotransmitter release regulation 1. The protein controls voltage-gated calcium channel recruitment to presynaptic membranes and modulates synaptic transmission, with balanced neurotransmission being critical for proper motor control 1. TSPOAP1 functions as a cytoplasmic protein closely associated with the mitochondrial transmembrane protein TSPO, participating in necroptosis and sirtuin signaling pathways, mitochondrial dysfunction, and inflammasome activation 2. Bioinformatic analyses suggest TSPOAP1 plays roles in inflammation and neuroinflammation through interactions with proteins like TNFR1, VCAM1, and NLRP3 2. Clinically, biallelic variants in TSPOAP1 cause autosomal recessive dystonia, with loss-of-function mutations leading to juvenile-onset generalized dystonia with intellectual disability and cerebellar atrophy, while missense variants cause adult-onset focal dystonia 1. The gene shows genetic associations with multiple neurodegenerative disorders including Alzheimer's disease and ALS, supporting neuroinflammation as a transdiagnostic mechanism 3. TSPOAP1 expression levels also serve as potential biomarkers for disease progression in spinocerebellar ataxia type 3 4.