Article ID Journal Published Year Pages File Type
2173561 Developmental Biology 2011 9 Pages PDF
Abstract

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.

► The consequences of expressing a mutant TRα1 receptor on cerebellum development are analyzed. ► Unliganded TRα1 is responsible for cerebellum defects in case of hypothyroidism.

Related Topics
Life Sciences Biochemistry, Genetics and Molecular Biology Cell Biology
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