Mast Cells Expressing the Germline HPS1 16-Bp Duplication (c.1470_1486dup16, Hermansky-Pudlak Syndrome-1) Defect Produce Extracellular Matrix Components

• Published in: Journal of allergy and clinical immunology Vol. 135; no. 2; p. AB172
• Main Authors: Kirshenbaum, Arnold S., MD, FAAAAI, Bandara, Geethani, PhD, Desai, Avanti, MS, Fischer, Elizabeth, PhD, Leerkes, Maarten, PhD, Metcalfe, Dean D., MD, Cruse, Glenn, PhD
• Format: Journal Article
• Language: English
• Published: St. Louis Elsevier Limited 01.02.2015
• Subjects: Allergy and Immunology; Cloning; Extracellular matrix; Pulmonary fibrosis

Rationale In the process of developing the Hermansky-Pudlak (HP) mastocyte (M) cell line from a patient with HPS-1, we noticed matrix production on culture flask surfaces which was reduced in clones transfected with wild type HPS1. Since human mast cells are associated with pulmonary fibrosis in pat...