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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
46
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pubmed:dateCreated |
2000-1-3
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pubmed:abstractText |
Fanconi anemia (FA) is a genetic disorder characterized by bone marrow failure, congenital abnormalities, cancer susceptibility, and a marked cellular hypersensitivity to DNA interstrand cross-linking agents, which correlates with a defect in ability to repair this type of damage. We have previously identified an approximately 230-kDa protein present in a nuclear protein complex in normal human lymphoblastoid cells that is involved in repair of DNA interstrand cross-links and shows reduced levels in FA-A cell nuclei. The FANCA gene appears to play a role in the stability or expression of this protein. We now show that p230 is a well known structural protein, human alpha spectrin II (alphaSpIISigma*), and that levels of alphaSpIISigma* are not only significantly reduced in FA-A cells but also in FA-B, FA-C and FA-D cells (i.e. in all FA cell lines tested), suggesting a role for these FA proteins in the stability or expression of alphaSpIISigma*. These studies also show that alphaSpIISigma* forms a complex in the nucleus with the FANCA and FANCC proteins. alphaSpIISigma* may thus act as a scaffold to align or enhance interactions between FA proteins and proteins involved in DNA repair. These results suggest that FA represents a disorder in which there is a deficiency in alphaSpIISigma*.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Cell Cycle Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/DNA-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/FANCC protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Fanconi Anemia Complementation...,
http://linkedlifedata.com/resource/pubmed/chemical/Fanconi Anemia Complementation...,
http://linkedlifedata.com/resource/pubmed/chemical/Nuclear Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Peptides,
http://linkedlifedata.com/resource/pubmed/chemical/Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Spectrin,
http://linkedlifedata.com/resource/pubmed/chemical/p 230
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pubmed:status |
MEDLINE
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pubmed:month |
Nov
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pubmed:issn |
0021-9258
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
12
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pubmed:volume |
274
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
32904-8
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:10551855-Blotting, Western,
pubmed-meshheading:10551855-Cell Cycle Proteins,
pubmed-meshheading:10551855-Cell Line,
pubmed-meshheading:10551855-DNA Repair,
pubmed-meshheading:10551855-DNA-Binding Proteins,
pubmed-meshheading:10551855-Fanconi Anemia,
pubmed-meshheading:10551855-Fanconi Anemia Complementation Group A Protein,
pubmed-meshheading:10551855-Fanconi Anemia Complementation Group C Protein,
pubmed-meshheading:10551855-Fanconi Anemia Complementation Group Proteins,
pubmed-meshheading:10551855-HeLa Cells,
pubmed-meshheading:10551855-Humans,
pubmed-meshheading:10551855-Nuclear Proteins,
pubmed-meshheading:10551855-Peptides,
pubmed-meshheading:10551855-Precipitin Tests,
pubmed-meshheading:10551855-Protein Binding,
pubmed-meshheading:10551855-Proteins,
pubmed-meshheading:10551855-Spectrin,
pubmed-meshheading:10551855-Spectrometry, Mass, Matrix-Assisted Laser...
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pubmed:year |
1999
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pubmed:articleTitle |
Human alpha spectrin II and the Fanconi anemia proteins FANCA and FANCC interact to form a nuclear complex.
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pubmed:affiliation |
Department of Pathology, University of Medicine and Dentistry-New Jersey Medical School, Newark, New Jersey 07103, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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