HPS6 is a subunit of the BLOC-2 (biogenesis of lysosomal organelles complex-2) protein complex that regulates lysosome-related organelle biogenesis and function 1. The protein acts as a cargo adapter linking lysosomes to the dynein-dynactin motor complex, mediating retrograde transport of lysosomes from the cell periphery to the perinuclear region 2. This perinuclear positioning is critical for lysosome maturation, acidification, degradative capacity, and fusion with late endosomes 2. HPS6 functions within a stable 340 kDa BLOC-2 complex alongside HPS3 and HPS5 proteins, with specific amino acid residues essential for complex assembly 13. The protein is particularly important for biogenesis of specialized organelles including melanosomes and platelet dense granules 1. Mutations in HPS6 cause Hermansky-Pudlak syndrome type 6 (HPS6), a rare autosomal recessive disorder characterized by oculocutaneous albinism, platelet dysfunction with deficient dense granules, and variable systemic manifestations including pulmonary fibrosis and immunodeficiency 456. HPS6 variants show clustering in CpG islands and specific protein structural regions, though no clear genotype-phenotype correlation has been established 6. Early molecular diagnosis via genomic sequencing is essential for clinical management 7.