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

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...

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Published inJournal 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
LanguageEnglish
Published St. Louis Elsevier Limited 01.02.2015
Subjects
Allergy and Immunology
Cloning
Extracellular matrix
Pulmonary fibrosis
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Summary: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 patients with HPS-1, HPM cells were examined for production of matrix components.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
ISSN:0091-6749
1097-6825
DOI:10.1016/j.jaci.2014.12.1497