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 in | Journal of allergy and clinical immunology Vol. 135; no. 2; p. AB172 |
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| Main Authors | , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
St. Louis
Elsevier Limited
01.02.2015
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| Abstract | 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. |
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| AbstractList | 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. |
| Author | Cruse, Glenn, PhD Bandara, Geethani, PhD Fischer, Elizabeth, PhD Leerkes, Maarten, PhD Kirshenbaum, Arnold S., MD, FAAAAI Desai, Avanti, MS Metcalfe, Dean D., MD |
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| SubjectTerms | Allergy and Immunology Cloning Extracellular matrix Pulmonary fibrosis |
| Title | Mast Cells Expressing the Germline HPS1 16-Bp Duplication (c.1470_1486dup16, Hermansky-Pudlak Syndrome-1) Defect Produce Extracellular Matrix Components |
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