BLOC1S6 and Hermansky-Pudlak syndrome: In humans, mutations in the genes encoding three BLOC-1 subunits, including dysbindin and pallidin, lead to a rare autosomal recessive disorder named Hermansky-Pudlak syndrome (HPS), which is characterised by deficient function of specialised lysosome-related organelles such as melanosomes and platelet dense granules with consequent presentations of albinism and prolonged bleeding times (reviewed by Wei, 2006; Huizing et al., 2008; Wei and Li, 2013; El-Chemaly and Young, 2016).