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| LUNG-DISEASE IN THE CYSTIC-FIBROSIS MOUSE EXPOSED TO BACTERIAL PATHOGENS |
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| Author(s): DAVIDSON DJ, DORIN JR, MCLACHLAN G, RANALDI V, LAMB D, DOHERTY C, GOVAN J, PORTEOUS DJ |
| Source: NATURE GENETICS Volume: 9 Issue: 4 Pages: 351-357 Published: APR 1995 |
| Times Cited: 93 References: 31 |
| Abstract: Lung disease is the major cause of death in cystic fibrosis (CF), but there is no evidence for overt lung involvement at birth. We show here that the same is true for the gene targeted cftr(m1HGU) mutant mouse. Furthermore, this CF mouse model demonstrates an impaired capacity to clear Staphylococcus aureus and Burkholderia (Pseudomonas) cepacia, two opportunistic lung pathogens closely associated with lung disease in CF subjects. The cftr(m1HGU) homozygotes display mucus retention and frank lung disease in response to repeated microbial exposure. Thus, lung disease in the cftr(m1HGU) mouse develops in response to bacterial infection, establishing a model to dissect the pathogenesis of CF pulmonary disease and providing a clinically relevant end point to assess the efficacy of pharmacologic or genetic interventions. |
| Document Type: Article |
| Language: English |
Addresses:
1. WESTERN GEN HOSP, MRC, HUMAN GENET UNIT, EDINBURGH EH4 2XU, MIDLOTHIAN SCOTLAND
2. WESTERN GEN HOSP, BIOMED RES FACIL, EDINBURGH EH4 2XU, MIDLOTHIAN SCOTLAND
3. UNIV EDINBURGH, SCH MED, DEPT PATHOL, EDINBURGH, MIDLOTHIAN SCOTLAND
4. UNIV EDINBURGH, SCH MED, DEPT MED MICROBIOL, EDINBURGH EH8 9AG, MIDLOTHIAN SCOTLAND |
| Publisher: NATURE PUBLISHING CO, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 |
| Subject Category: Genetics & Heredity |
| IDS Number: QQ753 |
| ISSN: 1061-4036 |
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