Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
17
pubmed:dateCreated
2003-4-21
pubmed:abstractText
p53-binding protein-1 (53BP1) is phosphorylated in response to DNA damage and rapidly relocalizes to presumptive sites of DNA damage along with Mre11 and the phosphorylated histone 2A variant, gamma-H2AX. 53BP1 associates with the BRCA1 tumor suppressor, and knock-down experiments with small interfering RNA have revealed a role for the protein in the checkpoint response to DNA damage. By generating mice defective in m53BP1 (m53BP1(tr/tr)), we have created an animal model to further explore its biochemical and genetic roles in vivo. We find that m53BP1(tr/tr) animals are growth-retarded and show various immune deficiencies including a specific reduction in thymus size and T cell count. Consistent with a role in responding to DNA damage, we find that m53BP1(tr/tr) mice are sensitive to ionizing radiation (gamma-IR), and cells from these animals exhibit chromosomal abnormalities consistent with defects in DNA repair. Thus, 53BP1 is a critical element in the DNA damage response and plays an integral role in maintaining genomic stability.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0021-9258
pubmed:author
pubmed:issnType
Print
pubmed:day
25
pubmed:volume
278
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
14971-7
pubmed:dateRevised
2011-11-17
pubmed:meshHeading
pubmed-meshheading:12578828-Active Transport, Cell Nucleus, pubmed-meshheading:12578828-Animals, pubmed-meshheading:12578828-Autoimmune Diseases, pubmed-meshheading:12578828-BRCA1 Protein, pubmed-meshheading:12578828-Carrier Proteins, pubmed-meshheading:12578828-Chromosomal Proteins, Non-Histone, pubmed-meshheading:12578828-Chromosome Aberrations, pubmed-meshheading:12578828-DNA Damage, pubmed-meshheading:12578828-DNA Repair, pubmed-meshheading:12578828-DNA-Binding Proteins, pubmed-meshheading:12578828-Disease Models, Animal, pubmed-meshheading:12578828-Genome, pubmed-meshheading:12578828-Growth, pubmed-meshheading:12578828-Intracellular Signaling Peptides and Proteins, pubmed-meshheading:12578828-Mice, pubmed-meshheading:12578828-Mice, Knockout, pubmed-meshheading:12578828-Phosphoproteins, pubmed-meshheading:12578828-Terminal Repeat Sequences
pubmed:year
2003
pubmed:articleTitle
Role for the BRCA1 C-terminal repeats (BRCT) protein 53BP1 in maintaining genomic stability.
pubmed:affiliation
Department of Biochemistry and Molecular Biology, University of Texas Health Sciences Center, Houston, Texas 77030, USA.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, U.S. Gov't, Non-P.H.S., Research Support, Non-U.S. Gov't