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Journal Article
Pitx2 regulates lung asymmetry, cardiac positioning and pituitary and tooth morphogenesis
1999
Overview
Pitx1
(refs
1
,
2
,
3
) and
Pitx2
(refs
4
,
5
) are highly homologous, bicoid-related transcription factors.
Pitx2
was initially identified as the gene responsible for the human Rieger syndrome
4
, an autosomal dominant condition that causes developmental abnormalities.
Pitx2
is asymmetrically expressed in the left lateral-plate mesoderm
5
,
6
,
7
,
8
,
9
,
10
,
11
, and mutant mice with laterality defects show altered patterns of
Pitx2
expression that correlate with changes in the visceral symmetry (
situs
). Ectopic expression of
Pitx2
in the right lateral-plate mesoderm alters looping of the heart and gut and reverses body rotation in chick and
Xenopus
embryos
6
,
7
,
8
,
9
,
10
,
11
. Here we describe the phenotype of
Pitx2
gene-deleted mice, characterized by defective body-wall closure, right pulmonary isomerism, altered cardiac position, arrest in turning and, subsequently, a block in the determination and proliferation events of anterior pituitary gland and tooth organogenesis. Thus,
Pitx2
is a transcription factor that encodes ‘leftness’ of the lung.
Publisher
Nature Publishing Group UK,Nature Publishing Group
Subject
Abnormalities, Multiple - embryology
/ Abnormalities, Multiple - genetics
/ Animals
/ Body Patterning - physiology
/ Chimera
/ Genes
/ Heart Defects, Congenital - embryology
/ Heart Defects, Congenital - genetics
/ Homeodomain Proteins - genetics
/ Homeodomain Proteins - physiology
/ Humanities and Social Sciences
/ letter
/ Lungs
/ Mutation
/ Paired Box Transcription Factors
/ Pituitary Gland - embryology
/ Receptors, Growth Factor - physiology
/ Rodents
/ Science
/ Transcription Factors - genetics
/ Transcription Factors - physiology
/ Xenopus
