A Human Progenitor Mast Cell Line for Allergic and Fibrotic Research and Therapeutic Screening
Hermansky-Pudlak Syndrome type-1 (HPS-1) is a rare genetic disorder that affects around 1 in 500,000 people worldwide and 1 in 1,800 Puerto Ricans. Patients with HPS-1 display oculocutaneous albinism, bleeding due to platelet abnormality, and pulmonary fibrosis. Those that develop pulmonary fibrosis often succumb and live no more than a decade after early onset of breathing problems.