BORCS8 is a core component of the BLOC-one-related complex (BORC), a multiprotein complex essential for lysosomal positioning and anterograde transport. As part of BORC, BORCS8 associates with the cytosolic face of lysosomes, where it recruits the small GTPase ARL8 and kinesin-1/-3 microtubule motors to promote lysosome movement toward the cell periphery in non-neuronal cells and the distal axon in neurons 1. This lysosomal redistribution is critical for neuronal development and function. Biallelic BORCS8 variants cause severe early-infantile neurodegenerative disorder characterized by global developmental delay, profound intellectual disability, hypotonia, limb spasticity, optic atrophy, leuko-axonopathy with hypomyelination, and supratentorial neurodegeneration 1. Pathogenic variants exhibit either reduced assembly with other BORC subunits or complete loss of BORC incorporation, resulting in impaired lysosome distribution to the cell periphery 1. Zebrafish studies confirm that borcs8 knockout recapitulates key disease features including decreased brain and eye size and neuromuscular anomalies 1. These findings establish BORCS8 deficiency as a distinct genetic cause of infantile-onset neurodegeneration and highlight the critical importance of BORC-mediated lysosome dynamics for central nervous system development and function 1.