Skip Navigation

This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
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 ISI Web of Science
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 arrow Search for citing articles in:
ISI Web of Science (56)
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Gould, T. D.
Right arrow Articles by Pfeifer, K.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Gould, T. D.
Right arrow Articles by Pfeifer, K.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Human Molecular Genetics, Vol 7, 483-487, Copyright © 1998 by Oxford University Press

ARTICLES

Imprinting of mouse Kvlqt1 is developmentally regulated

TD Gould and K Pfeifer
Laboratory of Mammalian Genes and Development, National Institute of Child Health and Human Development, Building 6B, Room 2B206, 9000 Rockville Pike, Bethesda, MD 20892, USA.

Mouse distal chromosome 7 contains a cluster of at least five imprinted genes. The syntenic region in humans, at 11p15.5, has been implicated in several genetic disorders. Consistent with the imprinted status of the genes in the region, Beckwith-Wiedemann syndrome (BWS) and Wilms tumor are each associated with loss of maternal information. Also mapping to 11p15.5 are long QT and Jervell and Lange-Nielsen (JLN) syndromes. In contrast to BWS and Wilms tumor, these syndromes do not show any parent of origin bias. Recently positional cloning has identified KVLQT1 as the 11p15.5 gene responsible for increased susceptibility to long QT and JLN syndromes. Other studies associate KVLQT1 with BWS. Human KVLQT1 is paternally imprinted in embryos. In this study we present a contig and transcript map of distal mouse 7 and we physically and genetically map mouse Kvlqt1 to the region. Mouse Kvlqt1 is strongly expressed in heart, lung, gut, kidney and uterus. While its early developmental expression is maternal in origin, the paternal allele becomes increasingly active during development. Late juvenile and adult animals show complete biallelism, suggesting an explanation for the lack of parent of origin bias in JLN and long QT.
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
 J. Physiol.Home page
G. C. L. Bett and R. L. Rasmusson
Modification of K+ channel-drug interactions by ancillary subunits
J. Physiol., February 15, 2008; 586(4): 929 - 950.
[Abstract] [Full Text] [PDF]

Home page
 Genome ResHome page
S. Jeong, Y. Hahn, Q. Rong, and K. Pfeifer
Accurate quantitation of allele-specific expression patterns by analysis of DNA melting
Genome Res., July 1, 2007; 17(7): 1093 - 1100.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Cell. Biol.Home page
Y. S. Yoon, S. Jeong, Q. Rong, K.-Y. Park, J. H. Chung, and K. Pfeifer
Analysis of the H19ICR Insulator
Mol. Cell. Biol., May 1, 2007; 27(9): 3499 - 3510.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
M. Imboden, H. Swan, I. Denjoy, I. M. Van Langen, P. J. Latinen-Forsblom, C. Napolitano, V. Fressart, G. Breithardt, M. Berthet, S. Priori, et al.
Female Predominance and Transmission Distortion in the Long-QT Syndrome
N. Engl. J. Med., December 28, 2006; 355(26): 2744 - 2751.
[Abstract] [Full Text] [PDF]

Home page
 J. Physiol.Home page
G. C. L. Bett, M. J. Morales, D. L. Beahm, M. E. Duffey, and R. L. Rasmusson
Ancillary subunits and stimulation frequency determine the potency of chromanol 293B block of the KCNQ1 potassium channel
J. Physiol., November 1, 2006; 576(3): 755 - 767.
[Abstract] [Full Text] [PDF]

Home page
 Genes Dev.Home page
D. Mancini-DiNardo, S. J.S. Steele, J. M. Levorse, R. S. Ingram, and S. M. Tilghman
Elongation of the Kcnq1ot1 transcript is required for genomic imprinting of neighboring genes.
Genes & Dev., May 15, 2006; 20(10): 1268 - 1282.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Endocrinol. Metab.Home page
T. Suzuki and K. Takimoto
Differential expression of Kv4 pore-forming and KChIP auxiliary subunits in rat uterus during pregnancy
Am J Physiol Endocrinol Metab, February 1, 2005; 288(2): E335 - E341.
[Abstract] [Full Text] [PDF]

Home page
 J. Med. Genet.Home page
K Nakabayashi, S Makino, S Minagawa, A C Smith, J S Bamforth, P Stanier, M Preece, L Parker-Katiraee, T Paton, M Oshimura, et al.
Genomic imprinting of PPP1R9A encoding neurabin I in skeletal muscle and extra-embryonic tissues
J. Med. Genet., August 1, 2004; 41(8): 601 - 608.
[Full Text] [PDF]

Home page
 J. Pharmacol. Exp. Ther.Home page
B. C. Knollmann, M. C. Casimiro, A. N. Katchman, S. G. Sirenko, T. Schober, Q. Rong, K. Pfeifer, and S. N. Ebert
Isoproterenol Exacerbates a Long QT Phenotype in Kcnq1-Deficient Neonatal Mice: Possible Roles for Human-Like Kcnq1 Isoform 1 and Slow Delayed Rectifier K+ Current
J. Pharmacol. Exp. Ther., July 1, 2004; 310(1): 311 - 318.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Cell. Biol.Home page
K.-Y. Park, E. A. Sellars, A. Grinberg, S.-P. Huang, and K. Pfeifer
The H19 Differentially Methylated Region Marks the Parental Origin of a Heterologous Locus without Gametic DNA Methylation
Mol. Cell. Biol., May 1, 2004; 24(9): 3588 - 3595.
[Abstract] [Full Text] [PDF]

Home page
 J CARDIOVASC PHARMACOL THERHome page
S. Wasson, H. K. Reddy, and M. L. Dohrmann
Current Perspectives of Electrical Remodeling and Its Therapeutic Implications
Journal of Cardiovascular Pharmacology and Therapeutics, April 1, 2004; 9(2): 129 - 144.
[Abstract] [PDF]

Home page
 Mol. Cell. Biol.Home page
Y. Wang, K. Joh, S. Masuko, H. Yatsuki, H. Soejima, A. Nabetani, C. V. Beechey, S. Okinami, and T. Mukai
The Mouse Murr1 Gene Is Imprinted in the Adult Brain, Presumably Due to Transcriptional Interference by the Antisense-Oriented U2af1-rs1 Gene
Mol. Cell. Biol., January 1, 2004; 24(1): 270 - 279.
[Abstract] [Full Text] [PDF]

Home page
 Genome ResHome page
H. Kiyosawa, I. Yamanaka, N. Osato, S. Kondo, and Y. Hayashizaki
Antisense Transcripts With FANTOM2 Clone Set and Their Implications for Gene Regulation
Genome Res., June 1, 2003; 13(6): 1324 - 1334.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
D. Mancini-DiNardo, S. J.S. Steele, R. S. Ingram, and S. M. Tilghman
A differentially methylated region within the gene Kcnq1 functions as an imprinted promoter and silencer
Hum. Mol. Genet., February 1, 2003; 12(3): 283 - 294.
[Abstract] [Full Text] [PDF]

Home page
 Genome ResHome page
H. Yatsuki, K. Joh, K. Higashimoto, H. Soejima, Y. Arai, Y. Wang, I. Hatada, Y. Obata, H. Morisaki, Z. Zhang, et al.
Domain Regulation of Imprinting Cluster in Kip2/Lit1 Subdomain on Mouse Chromosome 7F4/F5: Large-Scale DNA Methylation Analysis Reveals That DMR-Lit1 Is a Putative Imprinting Control Region
Genome Res., December 1, 2002; 12(12): 1860 - 1870.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
G. Mantovani, E. Ballare, E. Giammona, P. Beck-Peccoz, and A. Spada
The Gs{alpha} Gene: Predominant Maternal Origin of Transcription in Human Thyroid Gland and Gonads
J. Clin. Endocrinol. Metab., October 1, 2002; 87(10): 4736 - 4740.
[Abstract] [Full Text] [PDF]

Home page
 J. Physiol.Home page
M. Grunnet, T. Jespersen, H. B. Rasmussen, T. Ljungstrom, N. K Jorgensen, S.-P. Olesen, and D. A Klaerke
KCNE4 is an inhibitory subunit to the KCNQ1 channel
J. Physiol., July 1, 2002; 542(1): 119 - 130.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
C. Kanduri, G. Fitzpatrick, R. Mukhopadhyay, M. Kanduri, V. Lobanenkov, M. Higgins, and R. Ohlsson
A Differentially Methylated Imprinting Control Region within the Kcnq1 Locus Harbors a Methylation-sensitive Chromatin Insulator
J. Biol. Chem., May 10, 2002; 277(20): 18106 - 18110.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Cell. Biol.Home page
C. R. Kaffer, A. Grinberg, and K. Pfeifer
Regulatory Mechanisms at the Mouse Igf2/H19 Locus
Mol. Cell. Biol., December 1, 2001; 21(23): 8189 - 8196.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
L. S. Weinstein
The Stimulatory G Protein {alpha}-Subunit Gene: Mutations and Imprinting Lead to Complex Phenotypes
J. Clin. Endocrinol. Metab., October 1, 2001; 86(10): 4622 - 4626.
[Full Text] [PDF]

Home page
 Hum Mol GenetHome page
R. M. John, J. F. -X. Ainscough, S. C. Barton, and M. A. Surani
Distant cis-elements regulate imprinted expression of the mouse p57 Kip2 (Cdkn1c) gene: implications for the human disorder, Beckwith-Wiedemann syndrome
Hum. Mol. Genet., July 1, 2001; 10(15): 1601 - 1609.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
M. C. Casimiro, B. C. Knollmann, S. N. Ebert, J. C. Vary Jr., A. E. Greene, M. R. Franz, A. Grinberg, S. P. Huang, and K. Pfeifer
Targeted disruption of the Kcnq1 gene produces a mouse model of Jervell and Lange- Nielsen Syndrome
PNAS, February 27, 2001; 98(5): 2526 - 2531.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
S. Engemann, M. Strodicke, M. Paulsen, O. Franck, R. Reinhardt, N. Lane, W. Reik, and J. Walter
Sequence and functional comparison in the Beckwith-Wiedemann region: implications for a novel imprinting centre and extended imprinting
Hum. Mol. Genet., November 1, 2000; 9(18): 2691 - 2706.
[Abstract] [Full Text] [PDF]

Home page
 Genes Dev.Home page
C. R. Kaffer, M. Srivastava, K.-Y. Park, E. Ives, S. Hsieh, J. Batlle, A. Grinberg, S.-P. Huang, and K. Pfeifer
A transcriptional insulator at the imprinted H19/Igf2 locus
Genes & Dev., August 1, 2000; 14(15): 1908 - 1919.
[Abstract] [Full Text]

Home page
 Hum Mol GenetHome page
M. Paulsen, O. El-Maarri, S. Engemann, M. Strodicke, O. Franck, K. Davies, R. Reinhardt, W. Reik, and J. Walter
Sequence conservation and variability of imprinting in the Beckwith-Wiedemann syndrome gene cluster in human and mouse
Hum. Mol. Genet., July 22, 2000; 9(12): 1829 - 1841.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
N. J. Smilinich, C. D. Day, G. V. Fitzpatrick, G. M. Caldwell, A. C. Lossie, P. R. Cooper, A. C. Smallwood, J. A. Joyce, P. N. Schofield, W. Reik, et al.
A maternally methylated CpG island in KvLQT1 is associated with an antisense paternal transcript and loss of imprinting in Beckwith-Wiedemann syndrome
PNAS, July 6, 1999; 96(14): 8064 - 8069.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
K. Mitsuya, M. Meguro, M. P. Lee, M. Katoh, T. C. Schulz, H. Kugoh, M. A. Yoshida, N. Niikawa, A. P. Feinberg, and M. Oshimura
LIT1, an imprinted antisense RNA in the human KvLQT1 locus identified by screening for differentially expressed transcripts using monochromosomal hybrids
Hum. Mol. Genet., July 1, 1999; 8(7): 1209 - 1217.
[Abstract] [Full Text] [PDF]

Home page
 Cardiovasc ResHome page
D. M. Roden and S. Kupershmidt
From genes to channels: normal mechanisms
Cardiovasc Res, May 1, 1999; 42(2): 318 - 326.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
J. G. Falls, D. J. Pulford, A. A. Wylie, and R. L. Jirtle
Genomic Imprinting: Implications for Human Disease
Am. J. Pathol., March 1, 1999; 154(3): 635 - 647.
[Abstract] [Full Text] [PDF]

Home page
 Circ. Res.Home page
N. Neyroud, P. Richard, N. Vignier, C. Donger, I. Denjoy, L. Demay, M. Shkolnikova, R. Pesce, P. Chevalier, B. Hainque, et al.
Genomic Organization of the KCNQ1 K+ Channel Gene and Identification of C-Terminal Mutations in the Long-QT Syndrome
Circ. Res., February 19, 1999; 84(3): 290 - 297.
[Abstract] [Full Text] [PDF]

Home page
 Genes Dev.Home page
J. L. Thorvaldsen, K. L. Duran, and M. S. Bartolomei
Deletion of the H19 differentially methylated domain results in loss of imprinted expression of H19 and Igf2
Genes & Dev., December 1, 1998; 12(23): 3693 - 3702.
[Abstract] [Full Text]


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.