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Disruption of Fuz in mouse embryos generates hypoplastic hindbrain development and reduced cranial nerve ganglia.
Caiaffa, Carlo Donato; Ambekar, Yogeshwari S; Singh, Manmohan; Lin, Ying Linda; Wlodarczyk, Bogdan; Aglyamov, Salavat R; Scarcelli, Giuliano; Larin, Kirill V; Finnell, Richard H.
Afiliación
  • Caiaffa CD; Center for Precision Environmental Health, Baylor College of Medicine, Houston, Texas, USA.
  • Ambekar YS; Department of Pediatrics, Dell Pediatric Research Institute, University of Texas at Austin Dell Medical School, Austin, Texas, USA.
  • Singh M; Department of Biomedical Engineering, University of Houston, Houston, Texas, USA.
  • Lin YL; Department of Biomedical Engineering, University of Houston, Houston, Texas, USA.
  • Wlodarczyk B; Center for Precision Environmental Health, Baylor College of Medicine, Houston, Texas, USA.
  • Aglyamov SR; Center for Precision Environmental Health, Baylor College of Medicine, Houston, Texas, USA.
  • Scarcelli G; Department of Biomedical Engineering, University of Houston, Houston, Texas, USA.
  • Larin KV; Fischell Department of Bioengineering, University of Maryland, College Park, Maryland, USA.
  • Finnell RH; Department of Biomedical Engineering, University of Houston, Houston, Texas, USA.
Dev Dyn ; 2024 Mar 19.
Article en En | MEDLINE | ID: mdl-38501709
ABSTRACT

BACKGROUND:

The brain and spinal cord formation is initiated in the earliest stages of mammalian pregnancy in a highly organized process known as neurulation. Environmental or genetic interferences can impair neurulation, resulting in clinically significant birth defects known collectively as neural tube defects. The Fuz gene encodes a subunit of the CPLANE complex, a macromolecular planar polarity effector required for ciliogenesis. Ablation of Fuz in mouse embryos results in exencephaly and spina bifida, including dysmorphic craniofacial structures due to defective cilia formation and impaired Sonic Hedgehog signaling.

RESULTS:

We demonstrate that knocking Fuz out during embryonic mouse development results in a hypoplastic hindbrain phenotype, displaying abnormal rhombomeres with reduced length and width. This phenotype is associated with persistent reduction of ventral neuroepithelial stiffness in a notochord adjacent area at the level of the rhombomere 5. The formation of cranial and paravertebral ganglia is also impaired in these embryos.

CONCLUSIONS:

This study reveals that hypoplastic hindbrain development, identified by abnormal rhombomere morphology and persistent loss of ventral neuroepithelial stiffness, precedes exencephaly in Fuz ablated murine mutants, indicating that the gene Fuz has a critical function sustaining normal neural tube development and neuronal differentiation.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Dev Dyn Asunto de la revista: ANATOMIA Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Dev Dyn Asunto de la revista: ANATOMIA Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos