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| CORRECTION OF RADIATION SENSITIVITY IN ATAXIA-TELANGIECTASIA CELLS BY A TRUNCATED I-KAPPA-B-ALPHA |
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| Author(s): JUNG M, ZHANG Y, LEE S, DRITSCHILO A |
| Source: SCIENCE Volume: 268 Issue: 5217 Pages: 1619-1621 Published: JUN 16 1995 |
| Times Cited: 148 References: 31 |
| Abstract: Cells from patients with ataxia telangiectasia (AT) are hypersensitive to ionizing radiation and are defective in the regulation of DNA synthesis. A complementary DNA that corrects the radiation sensitivity and DNA synthesis defects in fibroblasts from an AT group D patient was isolated by expression cloning and shown to encode a truncated form of I kappa B-alpha, an inhibitor of the nuclear factor kappa B (NF-kappa B) transcriptional activator. The parental AT fibroblasts expressed large amounts of the I kappa B-alpha transcript and showed constitutive activation of NF-kappa B. The AT fibroblasts transfected with the truncated I kappa B-alpha expressed normal amounts of the I kappa B-alpha transcript and showed regulated activation of NF-kappa B. These results suggest that aberrant regulation of NF-kappa B and I kappa B-alpha contribute to the cellular defect in AT. |
| Document Type: Article |
| Language: English |
Addresses:
1. GEORGETOWN UNIV, SCH MED, DEPT RADIAT MED, WASHINGTON, DC 20007 USA
2. GEORGETOWN UNIV, SCH MED, DEPT IMMUNOL & MICROBIOL, WASHINGTON, DC 20007 USA |
| Publisher: AMER ASSOC ADVAN SCIENCE, 1333 H ST NW, WASHINGTON, DC 20005 |
| Subject Category: Multidisciplinary Sciences |
| IDS Number: RD459 |
| ISSN: 0036-8075 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |