Severe impairment of cerebellum development in mice expressing a dominant-negative mutation inactivating thyroid hormone receptor alpha1 isoform.

Teddy Fauquier, Eva Romero, Frédéric Picou, Fabrice Chatonnet, Xuan-Nhi Nguyen, Laure Quignodon, Frédéric Flamant

Université de Lyon, CNRS, INRA, Université Claude Bernard Lyon 1, École Normale, Supérieure de Lyon, Institut de Génomique Fonctionnelle de Lyon, France

Developmental biology 2011 Aug 15

filter terms:

Thyroid hormone deficiency is known to deeply affect cerebellum post-natal development. We present here a detailed analysis of the phenotype of a recently generated mouse model, expressing a dominant-negative TRα1 mutation. Although hormonal level is not affected, the cerebellum of these mice displays profound alterations in neuronal and glial differentiation, which are reminiscent of congenital hypothyroidism, indicating a predominant function of this receptor isoform in normal cerebellum development. Some of the observed effects might result from the cell autonomous action of the mutation, while others are more likely to result from a reduction in neurotrophic factor production. Copyright © 2011 Elsevier Inc. All rights reserved.

Citation

Teddy Fauquier, Eva Romero, Frédéric Picou, Fabrice Chatonnet, Xuan-Nhi Nguyen, Laure Quignodon, Frédéric Flamant. Severe impairment of cerebellum development in mice expressing a dominant-negative mutation inactivating thyroid hormone receptor alpha1 isoform. Developmental biology. 2011 Aug 15;356(2):350-8