RGD Reference Report - Pitx2-mediated cardiac outflow tract remodeling. - Rat Genome Database

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Pitx2-mediated cardiac outflow tract remodeling.

Authors: Ma, Hsiao-Yen  Xu, Jun  Eng, Diana  Gross, Michael K  Kioussi, Chrissa 
Citation: Ma HY, etal., Dev Dyn. 2013 May;242(5):456-68. doi: 10.1002/dvdy.23934. Epub 2013 Mar 12.
RGD ID: 12910561
Pubmed: PMID:23361844   (View Abstract at PubMed)
PMCID: PMC3673775   (View Article at PubMed Central)
DOI: DOI:10.1002/dvdy.23934   (Journal Full-text)


BACKGROUND: Heart morphogenesis involves sequential anatomical changes from a linear tube of a single channel peristaltic pump to a four-chamber structure with two channels controlled by one-way valves. The developing heart undergoes continuous remodeling, including septation.
RESULTS: Pitx2-null mice are characterized by cardiac septational defects of the atria, ventricles, and outflow tract. Pitx2-null mice also exhibited a short outflow tract, including unseptated conus and deformed endocardial cushions. Cushions were characterized with a jelly-like structure, rather than the distinct membrane-looking leaflets, indicating that endothelial mesenchymal transition was impaired in Pitx2(-/-) embryos. Mesoderm cells from the branchial arches and neural crest cells from the otic region contribute to the development of the endocardial cushions, and both were reduced in number. Members of the Fgf and Bmp families exhibited altered expression levels in the mutants.
CONCLUSIONS: We suggest that Pitx2 is involved in the cardiac outflow tract septation by promoting and/or maintaining the number and the remodeling process of the mesoderm progenitor cells. Pitx2 influences the expression of transcription factors and signaling molecules involved in the differentiation of the cushion mesenchyme during heart development.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
heart septal defect  ISOPitx2 (Mus musculus)12910561; 12910561 RGD 
heart septal defect  IMP 12910561 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Pitx2  (paired-like homeodomain 2)

Genes (Mus musculus)
Pitx2  (paired-like homeodomain transcription factor 2)

Genes (Homo sapiens)
PITX2  (paired like homeodomain 2)


Additional Information