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| The hMre11/hRad50 protein complex and Nijmegen breakage syndrome: Linkage of double-strand break repair to the cellular DNA damage response |
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| Author(s): Carney JP, Maser RS, Olivares H, Davis EM, Le Beau M, Yates JR, Hays L, Morgan WF, Petrini JHJ |
| Source: CELL Volume: 93 Issue: 3 Pages: 477-486 Published: MAY 1 1998 |
| Times Cited: 672 References: 67 |
| Abstract: Nijmegen breakage syndrome (NBS) is an autosomal recessive disorder characterized by increased cancer incidence, cell cycle checkpoint defects, and ionizing radiation sensitivity. We have isolated the gene encoding p95, a member of the hMre11/hRad50 double-strand break repair complex. The p95 gene mapped to 8q21.3, the region that contains the NBS locus, and p95 was absent from NBS cells established from NBS patients, p95 deficiency in these cells completely abrogates the formation of hMre11/hRad50 ionizing radiation-induced foci. Comparison of the p95 cDNA to the NBS1 cDNA indicated that the p95 gene and NBS1 are identical. The implication of hMre11/hRad50/p95 protein complex in NBS reveals a direct molecular link between DSB repair and cell cycle checkpoint functions. |
| Document Type: Article |
| Language: English |
| Reprint Address: Petrini, JHJ (reprint author), Univ Wisconsin, Sch Med, Genet Lab, Madison, WI 53706 USA |
Addresses:
1. Univ Wisconsin, Sch Med, Genet Lab, Madison, WI 53706 USA
2. Univ Calif San Francisco, Dept Radiat Oncol, San Francisco, CA 94143 USA
3. Univ Calif Berkeley, Lawrence Berkeley Lab, Div Life Sci, Berkeley, CA 94720 USA
4. Univ Washington, Dept Mol Biotechnol, Seattle, WA 98195 USA
5. Univ Chicago, Dept Med, Hematol Oncol Sect, Chicago, IL 60637 USA |
| Publisher: CELL PRESS, 1050 MASSACHUSETTES AVE, CIRCULATION DEPT, CAMBRIDGE, MA 02138 USA |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology |
| IDS Number: ZL787 |
| ISSN: 0092-8674 |
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