Jumonji, a nuclear protein that is necessary for normal heart development

Youngsook Lee, Alice J. Song, Robert Baker, Bruce Micales, Simon Conway, Gary E. Lyons

Research output: Contribution to journalArticle

106 Citations (Scopus)

Abstract

Jumonji (jmj) was cloned in a gene trap screen to identify and mutagenize genes important for heart development. To investigate the role of jmj in heart development, we generated mice homozygous for the jmj mutation. The jmj homozygous mouse embryos showed heart malformations, including ventricular septal defect, noncompaction of the ventricular wall, double- outlet right ventricle, and dilated atria. The jmj mutants died soon after birth, apparently as a result of respiratory insufficiency caused by rib and sternum defects in addition to the heart defects. In situ hybridization analyses suggested that cardiomyocytes were differentiated but developmental regulation of chamber-specific genes was defective in fetal hearts. Expression of jmj was detected in the myocardium, especially in the interventricular septum, ventricular wall, and outflow tract, which correlated well with the locations of defects observed in the hearts of mutant mice. Homozygous embryos failed to express the jmj transcript in all tissues except in the nervous system. Confocal microscopic examination using anti-JMJ antibodies indicated that the JMJ protein was localized in the nuclei of cells transfected with jmj. These data demonstrate that JMJ is a nuclear protein, which is essential for normal heart development and function.

Original languageEnglish (US)
Pages (from-to)932-938
Number of pages7
JournalCirculation Research
Volume86
Issue number9
StatePublished - May 12 2000
Externally publishedYes

Fingerprint

Nuclear Proteins
Embryonic Structures
Double Outlet Right Ventricle
Genes
Fetal Heart
Ventricular Septum
Sternum
Congenital Heart Defects
Ventricular Heart Septal Defects
Ribs
Cell Nucleus
Cardiac Myocytes
Respiratory Insufficiency
Nervous System
In Situ Hybridization
Anti-Idiotypic Antibodies
Myocardium
Parturition
Mutation
Proteins

Keywords

  • Cardiac abnormalities
  • Gene trap
  • Jumonji

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Cite this

Lee, Y., Song, A. J., Baker, R., Micales, B., Conway, S., & Lyons, G. E. (2000). Jumonji, a nuclear protein that is necessary for normal heart development. Circulation Research, 86(9), 932-938.

Jumonji, a nuclear protein that is necessary for normal heart development. / Lee, Youngsook; Song, Alice J.; Baker, Robert; Micales, Bruce; Conway, Simon; Lyons, Gary E.

In: Circulation Research, Vol. 86, No. 9, 12.05.2000, p. 932-938.

Research output: Contribution to journalArticle

Lee, Y, Song, AJ, Baker, R, Micales, B, Conway, S & Lyons, GE 2000, 'Jumonji, a nuclear protein that is necessary for normal heart development', Circulation Research, vol. 86, no. 9, pp. 932-938.
Lee Y, Song AJ, Baker R, Micales B, Conway S, Lyons GE. Jumonji, a nuclear protein that is necessary for normal heart development. Circulation Research. 2000 May 12;86(9):932-938.
Lee, Youngsook ; Song, Alice J. ; Baker, Robert ; Micales, Bruce ; Conway, Simon ; Lyons, Gary E. / Jumonji, a nuclear protein that is necessary for normal heart development. In: Circulation Research. 2000 ; Vol. 86, No. 9. pp. 932-938.
@article{0d4b4cca1563403baa411ad125861a6e,
title = "Jumonji, a nuclear protein that is necessary for normal heart development",
abstract = "Jumonji (jmj) was cloned in a gene trap screen to identify and mutagenize genes important for heart development. To investigate the role of jmj in heart development, we generated mice homozygous for the jmj mutation. The jmj homozygous mouse embryos showed heart malformations, including ventricular septal defect, noncompaction of the ventricular wall, double- outlet right ventricle, and dilated atria. The jmj mutants died soon after birth, apparently as a result of respiratory insufficiency caused by rib and sternum defects in addition to the heart defects. In situ hybridization analyses suggested that cardiomyocytes were differentiated but developmental regulation of chamber-specific genes was defective in fetal hearts. Expression of jmj was detected in the myocardium, especially in the interventricular septum, ventricular wall, and outflow tract, which correlated well with the locations of defects observed in the hearts of mutant mice. Homozygous embryos failed to express the jmj transcript in all tissues except in the nervous system. Confocal microscopic examination using anti-JMJ antibodies indicated that the JMJ protein was localized in the nuclei of cells transfected with jmj. These data demonstrate that JMJ is a nuclear protein, which is essential for normal heart development and function.",
keywords = "Cardiac abnormalities, Gene trap, Jumonji",
author = "Youngsook Lee and Song, {Alice J.} and Robert Baker and Bruce Micales and Simon Conway and Lyons, {Gary E.}",
year = "2000",
month = "5",
day = "12",
language = "English (US)",
volume = "86",
pages = "932--938",
journal = "Circulation Research",
issn = "0009-7330",
publisher = "Lippincott Williams and Wilkins",
number = "9",

}

TY - JOUR

T1 - Jumonji, a nuclear protein that is necessary for normal heart development

AU - Lee, Youngsook

AU - Song, Alice J.

AU - Baker, Robert

AU - Micales, Bruce

AU - Conway, Simon

AU - Lyons, Gary E.

PY - 2000/5/12

Y1 - 2000/5/12

N2 - Jumonji (jmj) was cloned in a gene trap screen to identify and mutagenize genes important for heart development. To investigate the role of jmj in heart development, we generated mice homozygous for the jmj mutation. The jmj homozygous mouse embryos showed heart malformations, including ventricular septal defect, noncompaction of the ventricular wall, double- outlet right ventricle, and dilated atria. The jmj mutants died soon after birth, apparently as a result of respiratory insufficiency caused by rib and sternum defects in addition to the heart defects. In situ hybridization analyses suggested that cardiomyocytes were differentiated but developmental regulation of chamber-specific genes was defective in fetal hearts. Expression of jmj was detected in the myocardium, especially in the interventricular septum, ventricular wall, and outflow tract, which correlated well with the locations of defects observed in the hearts of mutant mice. Homozygous embryos failed to express the jmj transcript in all tissues except in the nervous system. Confocal microscopic examination using anti-JMJ antibodies indicated that the JMJ protein was localized in the nuclei of cells transfected with jmj. These data demonstrate that JMJ is a nuclear protein, which is essential for normal heart development and function.

AB - Jumonji (jmj) was cloned in a gene trap screen to identify and mutagenize genes important for heart development. To investigate the role of jmj in heart development, we generated mice homozygous for the jmj mutation. The jmj homozygous mouse embryos showed heart malformations, including ventricular septal defect, noncompaction of the ventricular wall, double- outlet right ventricle, and dilated atria. The jmj mutants died soon after birth, apparently as a result of respiratory insufficiency caused by rib and sternum defects in addition to the heart defects. In situ hybridization analyses suggested that cardiomyocytes were differentiated but developmental regulation of chamber-specific genes was defective in fetal hearts. Expression of jmj was detected in the myocardium, especially in the interventricular septum, ventricular wall, and outflow tract, which correlated well with the locations of defects observed in the hearts of mutant mice. Homozygous embryos failed to express the jmj transcript in all tissues except in the nervous system. Confocal microscopic examination using anti-JMJ antibodies indicated that the JMJ protein was localized in the nuclei of cells transfected with jmj. These data demonstrate that JMJ is a nuclear protein, which is essential for normal heart development and function.

KW - Cardiac abnormalities

KW - Gene trap

KW - Jumonji

UR - http://www.scopus.com/inward/record.url?scp=0034640295&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034640295&partnerID=8YFLogxK

M3 - Article

VL - 86

SP - 932

EP - 938

JO - Circulation Research

JF - Circulation Research

SN - 0009-7330

IS - 9

ER -