Skip Navigation



Human Molecular Genetics Advance Access published online on September 30, 2004
Human Molecular Genetics, doi:10.1093/hmg/ddh314
© 2004 by Oxford University Press
This Article
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
13/23/2971    most recent
ddh314v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Chamberlain, S. J.
Right arrow Articles by Brannan, C. I.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Chamberlain, S. J.
Right arrow Articles by Brannan, C. I.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Article

Evidence for genetic modifiers of postnatal lethality in PWS-IC deletion mice

Stormy J. Chamberlain 1, Karen A. Johnstone 2, Amanda J. DuBose 2, Thomas A. Simon 2, Marisa S. Bartolomei 3, James L. Resnick 2*, and Camilynn I. Brannan 2

1 Department of Molecular Genetics and Microbiology, University of Florida College of Medicine, Box 100266, Gainesville, FL 32610; Department of Genetics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599
2 Department of Molecular Genetics and Microbiology, University of Florida College of Medicine, Box 100266, Gainesville, FL 32610
3 Howard Hughes Medical Institute and Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104

* To whom correspondence should be addressed. E-mail: resnick{at}mgm.ufl.edu.


  Abstract

Prader-Willi Syndrome (PWS), most notably characterized by infantile hypotonia, short stature and morbid obesity, results from deficiencies in multiple genes that are subject to genomic imprinting. The usefulness of current mouse models of PWS has been limited by neonatal lethality in affected mice. Here we report the survival of the PWS imprinting center (IC) deletion mice on a variety of strain backgrounds. Expression analyses of the genes affected in the PWS region suggest that while there is low-level expression from both parental alleles in PWS-IC deletion pups, this expression does not explain their survival on certain strain backgrounds. Rather, the data provides evidence for strain-specific modifier genes that support the survival of PWS-IC deletion mice.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Hum Mol GenetHome page
K. N. Leung, R. O. Vallero, A. J. DuBose, J. L. Resnick, and J. M. LaSalle
Imprinting regulates mammalian snoRNA-encoding chromatin decondensation and neuronal nucleolar size
Hum. Mol. Genet., November 15, 2009; 18(22): 4227 - 4238.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
C. M. Doe, D. Relkovic, A. S. Garfield, J. W. Dalley, D. E.H. Theobald, T. Humby, L. S. Wilkinson, and A. R. Isles
Loss of the imprinted snoRNA mbii-52 leads to increased 5htr2c pre-RNA editing and altered 5HT2CR-mediated behaviour
Hum. Mol. Genet., June 15, 2009; 18(12): 2140 - 2148.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
N. L.G. Miller, R. Wevrick, and P. L. Mellon
Necdin, a Prader-Willi syndrome candidate gene, regulates gonadotropin-releasing hormone neurons during development
Hum. Mol. Genet., January 15, 2009; 18(2): 248 - 260.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. M. Bischof, C. L. Stewart, and R. Wevrick
Inactivation of the mouse Magel2 gene results in growth abnormalities similar to Prader-Willi syndrome
Hum. Mol. Genet., November 15, 2007; 16(22): 2713 - 2719.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
K. A. Johnstone, A. J. DuBose, C. R. Futtner, M. D. Elmore, C. I. Brannan, and J. L. Resnick
A human imprinting centre demonstrates conserved acquisition but diverged maintenance of imprinting in a mouse model for Angelman syndrome imprinting defects
Hum. Mol. Genet., February 1, 2006; 15(3): 393 - 404.
[Abstract] [Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.