Abstract:
:Bloom syndrome (BS) is a rare autosomal recessive disorder characterized by growth deficiency, immunodeficiency, genomic instability, and the early development of cancers of many types. BLM, the protein encoded by BLM, the gene mutated in BS, is localized in nuclear foci and absent from BS cells. BLM encodes a DNA helicase, and proteins from three missense alleles lack displacement activity. BLM transfected into BS cells reduces the frequency of sister chromatid exchanges and restores BLM in the nucleus. Missense alleles fail to reduce the sister chromatid exchanges in transfected BS cells or restore the normal nuclear pattern. BLM complements a phenotype of a Saccharomyces cerevisiae sgs1 top3 strain, and the missense alleles do not. This work demonstrates the importance of the enzymatic activity of BLM for its function and nuclear localization pattern.
journal_name
Mol Biol Celljournal_title
Molecular biology of the cellauthors
Neff NF,Ellis NA,Ye TZ,Noonan J,Huang K,Sanz M,Proytcheva Mdoi
10.1091/mbc.10.3.665keywords:
subject
Has Abstractpub_date
1999-03-01 00:00:00pages
665-76issue
3eissn
1059-1524issn
1939-4586journal_volume
10pub_type
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