Kirshenbaum, Arnold (NIAID)
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.
Scientists at the National Institute of Allergy and Infectious Diseases (NIAID) have developed the HPS-1 proMastocyte (HPM) cell line, containing an HPS-1 mutation. This cell line resembles a progenitor mast cell with reduced granule formation, significant chemotactic ability, and is the first mast cell line shown to constitutively release cytokines, chemokines, and most importantly fibrotic proteins. This cell line serves as a model to study granule formation, early mast cell development, chemotaxis and mechanisms controlling synthesis of molecules contributing to fibrosis.
Cell line available as live cells approximately 3-4 million cells per sample in a T25 Flask.
- A tool to further understand fibrosis
- A tool to study granule formation, early mast cell development, degranulation and chemotaxis
- Screening tool to identify target compounds for the treatment of pulmonary fibrosis
- First progenitor mast cell line known to produce fibrotic elements
- Progenitor mast cell line with rapid growth, no cytokine stimulation needed. Cell doubling time of 2–3 days