| | |  | | | | Record from Web of Science® | | | | | | |
| PATHOGENESIS OF PULMONARY FIBROSIS - PLATELET-DERIVED GROWTH-FACTOR PRECEDES STRUCTURAL ALTERATIONS IN THE HERMANSKY-PUDLAK SYNDROME |
|
|
| Author(s): HARMON KR, WITKOP CJ, WHITE JG, KING RA, PETERSON M, MOORE D, TASHJIAN J, MARINELLI WA, BITTERMAN PB |
| Source: JOURNAL OF LABORATORY AND CLINICAL MEDICINE Volume: 123 Issue: 4 Pages: 617-627 Published: APR 1994 |
| Times Cited: 45 References: 57 |
| Abstract: Peptides that modulate mesenchymal cell function have been detected in the fibrotic lung disorders once physiologic dysfunction is present. Despite this close association with manifest disease, their role in initiating alveolar remodeling remains unknown. We examined the hypothesis that one potent peptide, platelet-derived growth factor (PDGF), would be present at the alveolar surface before the onset of physiologic dysfunction in patients in whom pulmonary fibrosis subsequently develops. Bronchoalveolar ravage and physiologic assessment were performed in asymptomatic patients with the Hermansky-Pudlak syndrome (n = 30), obligate heterozygous (n = 9), and normal volunteers (control group). Lavage cell number and profile were normal, but alveolar macrophages demonstrated characteristic autofluorescence and ultrastructural features of ceroid. Lavage fluid from physiologically normal patients with Hermansky-Pudlak syndrome and from those with occult restrictive disease demonstrated two PDGF-related peptides (14 kd and 38 kd). Radioligand binding and fibroblast proliferation assay demonstrated that the peptides were functional. By immunoassay the concentration of PDGF in lavage fluid was six times greater than control values (p < 0.01). In situ hybridization together with bioassay indicated that alveolar macrophages were one cellular source of PDGF. Similar results were obtained for heterozygotes. These data identify macrophage-derived PDGF peptides as important candidate molecules in the initiation of alveolar remodeling in the fibrotic lung disorders. |
| Document Type: Article |
| Language: English |
| Reprint Address: HARMON, KR (reprint author), PARK NICOLLET MED CTR, DEPT MED, MINNEAPOLIS, MN USA |
Addresses:
1. UNIV MINNESOTA, SCH MED, DEPT MED, MINNEAPOLIS, MN 55455 USA
2. UNIV MINNESOTA, SCH MED, DEPT LAB MED, MINNEAPOLIS, MN 55455 USA
3. UNIV MINNESOTA, SCH MED, DEPT ORAL SCI, MINNEAPOLIS, MN 55455 USA
4. UNIV MINNESOTA, SCH MED, DEPT RADIOL, MINNEAPOLIS, MN 55455 USA |
| Publisher: MOSBY-YEAR BOOK INC, 11830 WESTLINE INDUSTRIAL DR, ST LOUIS, MO 63146-3318 |
| Subject Category: Medical Laboratory Technology; Medicine, General & Internal; Medicine, Research & Experimental |
| IDS Number: NE676 |
| ISSN: 0022-2143 |
|
| | | | | | | | | Record from Web of Science® | | | | | | | | | |