Quantification of gene expression patterns to reveal the origins of abnormal morphogenesis

The earliest developmental origins of dysmorphologies are poorly understood in many congenital diseases. They often remain elusive because the first signs of genetic misregulation may initiate as subtle changes in gene expression, which are hard to detect and can be obscured later in development by...

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Detalles Bibliográficos
Autores: Martinez-Abadias, Neus, Mateu Estivill, Roger, Sastre Tomas, Jaume, Perrine, Susan Motch, Yoon, Melissa, Robert-Moreno, Alexandre, Swoger, Jim, Russo, Lucia, Kawasaki, Kazuhiko, Richtsmeier, Joan, Sharpe, James
Tipo de recurso: artículo
Fecha de publicación:2018
País:España
Institución:Conselleria de Salut i Consum del Govern de les Illes Balears
Repositorio:Docusalut
Idioma:inglés
OAI Identifier:oai:docusalut.com:20.500.13003/9120
Acceso en línea:https://hdl.handle.net/20.500.13003/9120
Access Level:acceso abierto
Palabra clave:Mice, Inbred C57BL
Acrocephalosyndactylia
Tomography, X-Ray Computed
Receptor, Fibroblast Growth Factor, Type 2
Animals
Gene Expression Profiling
Gene Expression Regulation, Developmental
Biometry
Disease Models, Animal
Mutation, Missense
Receptor Tipo 2 de Factor de Crecimiento de Fibroblastos
Animales
Tomografía Computarizada por Rayos X
Mutación Missense
Acrocefalosindactilia
Ratones Endogámicos C57BL
Biometría
Regulación del Desarrollo de la Expresión Génica
Perfilación de la Expresión Génica
Modelos Animales de Enfermedad
Descripción
Sumario:The earliest developmental origins of dysmorphologies are poorly understood in many congenital diseases. They often remain elusive because the first signs of genetic misregulation may initiate as subtle changes in gene expression, which are hard to detect and can be obscured later in development by secondary effects. Here, we develop a method to trace back the origins of phenotypic abnormalities by accurately quantifying the 3D spatial distribution of gene expression domains in developing organs. By applying Geometric Morphometrics to 3D gene expression data obtained by Optical Projection Tomography, we determined that our approach is sensitive enough to find regulatory abnormalities that have never been detected previously. We identified subtle but significant differences in the gene expression of a downstream target of a Fgfr2 mutation associated with Apert syndrome, demonstrating that these mouse models can further our understanding of limb defects in the human condition. Our method can be applied to different organ systems and models to investigate the etiology of malformations.