Congenital hypothyroid Pax8-/- mutant mice can be rescued by inactivating the TRα gene
Congenital hypothyroid Pax8-/- mutant mice can be rescued by inactivating the TRα gene
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DOI：
10.1210/me.16.1.24
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发表时间：
2002-01-01
影响因子：
--
通讯作者：
Samarut, J
中科院分区：
文献类型：
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作者：
Flamant, F;Poguet, AL;Samarut, J
缺乏所有TRs的小鼠是存活的，而Pax8(-/-)小鼠在出生后的第一周就会死亡，因为Pax8(-/-)小鼠在甲状腺中缺乏产生T-4和T-3的滤泡细胞。对这两种突变的精确比较表明，它们的表型相似，但脾、骨和小肠的缺陷在Pax(-/-)小鼠中更为明显。这被解释为未连接的tr对Pax(-/-)突变体中甲状腺激素靶基因表达的负面影响的结果。Pax8(-/-)复合突变体可以存活到成年，目的基因的表达部分恢复。这证明了trα脱辅基受体活性在出生后发育的几个方面的重要性。
Mice devoid of all TRs are viable, whereas Pax8(-/-)mice, which lack the follicular cells producing T-4 and T-3 in the thyroid gland, die during the first weeks of postnatal life. A precise comparison between the two types of mutants reveals that their phenotypes are similar, but the defects in spleen, bone, and small intestine are more pronounced in Pax(-/-) mice. This is interpreted as the result of a negative effect of the unliganded TR on thyroid hormone target genes expression in the Pax(-/-)mutants. Pax8(-/-) compound mutants can survive to adulthood, and the expression of target genes is partially restored. This demonstrates the importance of TR alpha aporeceptor activity in several aspects of postnatal development.