| POT of gold: modeling dyskeratosis congenita in the mouse. | |
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MedLine Citation:
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PMID: 18593874 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Dyskeratosis congenita (DC) is a rare syndrome, characterized by cutaneous abnormalities and premature death caused by bone marrow failure. In this issue of Genes & Development, Hockemeyer and colleagues (pp. 1773-1785) report a new mouse model that reconstitutes key features of DC. Disease phenotypes are generated by a POT1b deletion in a telomerase-deficient background that accelerates the shortening of telomeres by degradation. |
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Authors:
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Chantal Autexier |
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Publication Detail:
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Type: Comment; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Genes & development Volume: 22 ISSN: 0890-9369 ISO Abbreviation: Genes Dev. Publication Date: 2008 Jul |
Date Detail:
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Created Date: 2008-07-02 Completed Date: 2008-09-10 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 8711660 Medline TA: Genes Dev Country: United States |
Other Details:
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Languages: eng Pagination: 1731-6 Citation Subset: IM |
Affiliation:
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Department of Anatomy and Cell Biology and Department of Medicine, McGill University, Montreal, Quebec H3T 1E2, Canada. chantal.autexier@mcgill.ca |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Bone Marrow / abnormalities, metabolism DNA-Binding Proteins / genetics, physiology* Disease Models, Animal Dyskeratosis Congenita* / genetics, pathology Mice Skin Pigmentation Telomerase / genetics, metabolism* Telomere / ultrastructure* |
| Chemical | |
Reg. No./Substance:
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0/DNA-Binding Proteins; 0/POT1b protein, mouse; EC 2.7.7.49/Telomerase |
| Comments/Corrections | |
Comment On:
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Genes Dev. 2008 Jul 1;22(13):1773-85
[PMID:
18550783
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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