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| GENERATION AND CHARACTERIZATION OF A DELTA-F508 CYSTIC-FIBROSIS MOUSE MODEL |
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| Author(s): COLLEDGE WH, ABELLA BS, SOUTHERN KW, RATCLIFF R, JIANG CW, CHENG SH, MACVINISH LJ, ANDERSON JR, CUTHBERT AW, EVANS MJ |
| Source: NATURE GENETICS Volume: 10 Issue: 4 Pages: 445-452 Published: AUG 1995 |
| Times Cited: 128 References: 43 |
| Abstract: We have generated mice carrying the most common mutation in cystic fibrosis (CF), Delta F508, within the cystic fibrosis (Cftr) gene. Mutant animals show pathological and electrophysiological changes consistent with a CF phenotype. Delta F508(-/-) mice die from peritonitis and show deficiencies in cAMP-activated electrogenic Cl- transport. These mice produce Delta F508 transcripts and show the temperature-dependent trafficking defect first described for the human Delta F508 CFTR protein. A functional CFTR Cl- channel not demonstrated by null CF mice or present at 37 degrees C was detected following incubation of epithelial cells at 27 degrees C. Thus, these mice are an accurate Delta F508 model and will be valuable for testing drugs aimed at overcoming the Delta F508 trafficking defect. |
| Document Type: Article |
| Language: English |
Addresses:
1. UNIV CAMBRIDGE, WELLCOME INST CANC & DEV BIOL, CRC, CAMBRIDGE CB2 1QR, ENGLAND
2. UNIV CAMBRIDGE, DEPT GENET, CAMBRIDGE CB2 1QR, ENGLAND
3. UNIV CAMBRIDGE, DEPT PHARMACOL, CAMBRIDGE CB2 1QJ, ENGLAND
4. GENZYME CORP, FRAMINGHAM, MA 01710 USA
5. ADDENBROOKES HOSP, DEPT HISTOPATHOL, CAMBRIDGE CB2 2QQ, ENGLAND |
| Publisher: NATURE PUBLISHING CO, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 |
| Subject Category: Genetics & Heredity |
| IDS Number: RM375 |
| ISSN: 1061-4036 |
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