The AP-3 complex is required for the endosomal trafficking of a subset of cargo to lysosomes and lysosome-related organelles (LROs), and mutations in AP3B1 subunit are associated with Hermansky-Pudlak Syndrome (HPS), characterized by lysosomal LRO abnormalities leading to albinism, clotting abnormalities, and pulmonary as well as renal dysfunction32, 34. Here, AP3B1 is linked to albinism.