TMEM230 encodes a transmembrane protein involved in synaptic vesicle trafficking and recycling within the endomembrane system. 1 The protein localizes to secretory and recycling vesicles and functions in intracellular vesicle transport, including trafficking through the trans-Golgi network, early endosomes, and recycling endosomes. 2 Recent structural studies reveal TMEM230 operates as a subunit of ATP8 and ATP11 lipid flippases on early endosomes, 2 and may regulate motor protein-mediated trafficking of metalloproteins and cellular components critical for neural tissue homeostasis. 3 TMEM230 mutations cause autosomal dominant Parkinson's disease with typical Lewy body pathology and late-onset presentation. 4 1 Pathogenic variants including p.Y92C, p.R141L, and frameshift mutations have been identified, though mutation prevalence remains rare and replication studies have been largely negative. 5 4 Despite limited genetic prevalence, TMEM230 represents a putative novel Parkinson's disease gene, 6 and patients with TMEM230 mutations exhibit significantly earlier disease onset (median ~31.5 years) compared to non-carriers. 7 The functional link between TMEM230 dysfunction and neurodegeneration remains incompletely understood. Current evidence suggests involvement in vesicle trafficking, autophagy, and protein aggregation pathways relevant to α-synuclein pathology, 5 though mechanistic studies characterizing how mutations lead to dopaminergic neuronal loss require further investigation.