COUP-TF Genes, Human Diseases, and the Development of the Central Nervous System in Murine Models.
Curr Top Dev Biol
; 125: 275-301, 2017.
Article
em En
| MEDLINE
| ID: mdl-28527575
ABSTRACT
COUP-TFI and -TFII are members of the steroid/thyroid nuclear receptor superfamily. Recent clinical studies reveal that COUP-TFI gene mutations are associated with Bosch-Boonstra-Schaaf optic atrophy syndrome displaying symptoms of optic atrophy, intellectual disability, hypotonia, seizure, autism spectrum disorders, oromotor dysfunction, thin corpus callosum, or hearing defects, and COUP-TFII gene mutations lead to congenital heart defects and/or congenital diaphragmatic hernia with developmental delay and mental defects. In this review, we first describe the functions of COUP-TF genes in the morphogenesis of mouse forebrain including cerebral cortex, hippocampus, amygdala complex, hypothalamus, and cortical interneuron. Then, we address their roles in the development of cerebellum, glial cells, neural crest cells, and adult neuronal stem cells. Clearly, the investigations on the functions of COUP-TF genes in the developing mouse central nervous system will benefit not only the understanding of neurodevelopment, but also the etiology of human mental diseases.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Sistema Nervoso Central
/
Regulação da Expressão Gênica no Desenvolvimento
/
Fatores de Transcrição COUP
Tipo de estudo:
Prognostic_studies
Limite:
Animals
/
Humans
Idioma:
En
Revista:
Curr Top Dev Biol
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
China