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PMID:11309417

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Citation

Bischof, O, Kim, SH, Irving, J, Beresten, S, Ellis, NA and Campisi, J (2001) Regulation and localization of the Bloom syndrome protein in response to DNA damage. J. Cell Biol. 153:367-80

Abstract

Bloom syndrome (BS) is an autosomal recessive disorder characterized by a high incidence of cancer and genomic instability. BLM, the protein defective in BS, is a RecQ-like helicase, presumed to function in DNA replication, recombination, or repair. BLM localizes to promyelocytic leukemia protein (PML) nuclear bodies and is expressed during late S and G2. We show, in normal human cells, that the recombination/repair proteins hRAD51 and replication protein (RP)-A assembled with BLM into a fraction of PML bodies during late S/G2. Biochemical experiments suggested that BLM resides in a nuclear matrix-bound complex in which association with hRAD51 may be direct. DNA-damaging agents that cause double strand breaks and a G2 delay induced BLM by a p53- and ataxia-telangiectasia mutated independent mechanism. This induction depended on the G2 delay, because it failed to occur when G2 was prevented or bypassed. It coincided with the appearance of foci containing BLM, PML, hRAD51 and RP-A, which resembled ionizing radiation-induced foci. After radiation, foci containing BLM and PML formed at sites of single-stranded DNA and presumptive repair in normal cells, but not in cells with defective PML. Our findings suggest that BLM is part of a dynamic nuclear matrix-based complex that requires PML and functions during G2 in undamaged cells and recombinational repair after DNA damage.

Links

PubMed PMC2169463

Keywords

Adenosine Triphosphatases/genetics; Adenosine Triphosphatases/metabolism; Bloom Syndrome/genetics; Blotting, Western; Cell Cycle/physiology; Cell Fractionation; Cell Nucleus/metabolism; Cells, Cultured; DNA Damage; DNA Helicases/genetics; DNA Helicases/metabolism; DNA Repair/physiology; DNA-Binding Proteins/metabolism; Fibroblasts/metabolism; Fibroblasts/radiation effects; Flow Cytometry; Humans; Microscopy, Fluorescence; Neoplasm Proteins/metabolism; Nuclear Proteins/genetics; Nuclear Proteins/metabolism; Proteins/metabolism; Rad51 Recombinase; RecQ Helicases; Replication Protein A; Transcription Factors/metabolism; Tubulin/metabolism; Tumor Suppressor Proteins; X-Rays

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:BLM

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:Q06609

F

Seeded From UniProt

complete

HUMAN:BLM

located_in

GO:0016363: nuclear matrix

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:BLM

involved_in

GO:0007095: mitotic G2 DNA damage checkpoint signaling

ECO:0000314: direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:RAD51

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:P54132

F

Seeded From UniProt

complete

HUMAN:RAD51

located_in

GO:0016605: PML body

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:BLM

involved_in

GO:0007095: mitotic G2 DNA damage checkpoint

ECO:0000314: direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:BLM

involved_in

GO:0000724: double-strand break repair via homologous recombination

ECO:0000303: author statement without traceable support used in manual assertion

P

Seeded From UniProt

complete

HUMAN:BLM

part_of

GO:0016363: nuclear matrix

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:BLM

involved_in

GO:0010165: response to X-ray

ECO:0000314: direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:RAD51

part_of

GO:0016605: PML body

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete


See also

References

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