PUBLICATION

Nkx2.7 is a conserved regulator of craniofacial development

Authors
Ford, C., de Sena-Tomás, C., Wun, T.T.R., Aleman, A.G., Rangaswamy, U., Leyhr, J., Nuñez, M.I., Gao, C.Z., Nim, H.T., See, M., Coppola, U., Waxman, J.S., Ramialison, M., Haitina, T., Smeeton, J., Sanges, R., Targoff, K.L.
ID
ZDB-PUB-250424-13
Date
2025
Source
Nature communications   16: 38023802 (Journal)
Registered Authors
Coppola, Ugo, Haitina, Tatjana, Leyhr, Jake, Ramialison, Mirana, Smeeton, Joanna, Targoff, Kimara, Waxman, Joshua
Keywords
none
Datasets
GEO:GSE240780
MeSH Terms
  • Signal Transduction
  • Receptors, Notch/metabolism
  • Endothelin-1/genetics
  • Endothelin-1/metabolism
  • Transcription Factors*/genetics
  • Transcription Factors*/metabolism
  • Animals
  • Zebrafish/embryology
  • Zebrafish/genetics
  • Mice
  • Skull*/embryology
  • Neural Crest/embryology
  • Neural Crest/metabolism
  • Homeodomain Proteins*/genetics
  • Homeodomain Proteins*/metabolism
  • Homeobox Protein Nkx-2.5/genetics
  • Homeobox Protein Nkx-2.5/metabolism
  • Branchial Region/metabolism
  • Gene Expression Regulation, Developmental
  • Craniofacial Abnormalities/genetics
  • Evolution, Molecular
  • Zebrafish Proteins*/genetics
  • Zebrafish Proteins*/metabolism
PubMed
40268889 Full text @ Nat. Commun.
Abstract
Craniofacial malformations arise from developmental defects in the head, face, and neck with phenotypes such as 22q11.2 deletion syndrome illustrating a developmental link between cardiovascular and craniofacial morphogenesis. NKX2-5 is a key cardiac transcription factor associated with congenital heart disease and mouse models of Nkx2-5 deficiency highlight roles in cardiac development. In zebrafish, nkx2.5 and nkx2.7 are paralogues in the NK4 family expressed in cardiomyocytes and pharyngeal arches. Despite shared cellular origins of cardiac and craniofacial tissues, the function of NK4 factors in head and neck patterning has not been elucidated. Molecular evolutionary analysis of NK4 genes shows that nkx2.5 and nkx2.7 are ohnologs resulting from whole genome duplication events. Nkx2.7 serves as a previously unappreciated regulator of branchiomeric muscle and cartilage formation for which nkx2.5 cannot fully compensate. Mechanistically, our results highlight that Nkx2.7 patterns the cranial neural crest and functions upstream of Endothelin1 to inhibit Notch signals. Together, our studies shed light on an evolutionarily conserved Nkx transcription factor with unique functions in vertebrate craniofacial development, advancing our understanding of congenital head and neck deformities.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping