Chromosome instability in fibroblasts derived from Li-Fraumeni syndrome families without TP53 mutations.

2.50
Hdl Handle:
http://hdl.handle.net/10541/86214
Title:
Chromosome instability in fibroblasts derived from Li-Fraumeni syndrome families without TP53 mutations.
Authors:
Boyle, John M; Spreadborough, Anne R; Greaves, M J; Birch, Jillian M; Scott, David
Abstract:
The mean in vitro lifespan of dermal fibroblast strains derived from cancer-affected individuals belonging to families conforming to the classical Li-Fraumeni-syndrome or the Li-Fraumeni-like syndrome (LF strains), but in whom no TP53 mutation has been found, was not significantly different to that of normal strains. This was in contrast to LF strains that carry TP53 mutations. Cytogenetic observations of numerical and structural chromosome abnormalities were made on Giemsa stained metaphases prepared at different times during the lifespan of strains. Five strains from different LF families showed significantly increased frequencies of abnormal cells during the last 10% of their lifetime compared with seven normal strains and three other LF strains fell outside the normal range but did not reach significance. Two LF strains fell within the normal range indicating heterogeneity of the phenotype in this subset of LF fibroblasts. Numerical aberrations were the major aberration type observed. These observations of genetic instability are similar, but generally less strongly expressed, to those seen in LF strains with TP53 mutations. The basis for genetic instability in LF strains without TP53 mutations is not known, but appears not to involve defects in either the G(1)checkpoint or the checkpoint kinase hChk2.
Affiliation:
CRC Cancer Genetics Group, Paterson Institute for Cancer Research, CRC Christie Research Centre, Manchester, UK.
Citation:
Chromosome instability in fibroblasts derived from Li-Fraumeni syndrome families without TP53 mutations. 2000, 83 (9):1136-8 Br. J. Cancer
Journal:
British Journal of Cancer
Issue Date:
Nov-2000
URI:
http://hdl.handle.net/10541/86214
DOI:
10.1054/bjoc.2000.1444
PubMed ID:
11027425
Type:
Article
Language:
en
ISSN:
0007-0920
Appears in Collections:
All Paterson Institute for Cancer Research

Full metadata record

DC FieldValue Language
dc.contributor.authorBoyle, John Men
dc.contributor.authorSpreadborough, Anne Ren
dc.contributor.authorGreaves, M Jen
dc.contributor.authorBirch, Jillian Men
dc.contributor.authorScott, Daviden
dc.date.accessioned2009-11-13T16:25:58Z-
dc.date.available2009-11-13T16:25:58Z-
dc.date.issued2000-11-
dc.identifier.citationChromosome instability in fibroblasts derived from Li-Fraumeni syndrome families without TP53 mutations. 2000, 83 (9):1136-8 Br. J. Canceren
dc.identifier.issn0007-0920-
dc.identifier.pmid11027425-
dc.identifier.doi10.1054/bjoc.2000.1444-
dc.identifier.urihttp://hdl.handle.net/10541/86214-
dc.description.abstractThe mean in vitro lifespan of dermal fibroblast strains derived from cancer-affected individuals belonging to families conforming to the classical Li-Fraumeni-syndrome or the Li-Fraumeni-like syndrome (LF strains), but in whom no TP53 mutation has been found, was not significantly different to that of normal strains. This was in contrast to LF strains that carry TP53 mutations. Cytogenetic observations of numerical and structural chromosome abnormalities were made on Giemsa stained metaphases prepared at different times during the lifespan of strains. Five strains from different LF families showed significantly increased frequencies of abnormal cells during the last 10% of their lifetime compared with seven normal strains and three other LF strains fell outside the normal range but did not reach significance. Two LF strains fell within the normal range indicating heterogeneity of the phenotype in this subset of LF fibroblasts. Numerical aberrations were the major aberration type observed. These observations of genetic instability are similar, but generally less strongly expressed, to those seen in LF strains with TP53 mutations. The basis for genetic instability in LF strains without TP53 mutations is not known, but appears not to involve defects in either the G(1)checkpoint or the checkpoint kinase hChk2.en
dc.language.isoenen
dc.subjectTumour Suppressor Protein p53en
dc.subject.meshAdolescent-
dc.subject.meshAdult-
dc.subject.meshAged-
dc.subject.meshAneuploidy-
dc.subject.meshChromosome Aberrations-
dc.subject.meshFamily Health-
dc.subject.meshFemale-
dc.subject.meshFibroblasts-
dc.subject.meshHumans-
dc.subject.meshKaryotyping-
dc.subject.meshLi-Fraumeni Syndrome-
dc.subject.meshMale-
dc.subject.meshMiddle Aged-
dc.subject.meshMutation-
dc.subject.meshTumor Suppressor Protein p53-
dc.titleChromosome instability in fibroblasts derived from Li-Fraumeni syndrome families without TP53 mutations.en
dc.typeArticleen
dc.contributor.departmentCRC Cancer Genetics Group, Paterson Institute for Cancer Research, CRC Christie Research Centre, Manchester, UK.en
dc.identifier.journalBritish Journal of Canceren

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