Very strong correlation between dominant negative activities of mutant thyroid hormone receptors and their binding avidity for corepressor SMRT

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Regulation and binding of pregnane X receptor by nuclear receptor corepressor silencing mediator of retinoid and thyroid hormone receptors (SMRT).

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Dominant Negative Activity of Mutant Thyroid Hormone α1 Receptors from Patients with Hepatocellular Carcinoma.

Complementary DNAs for two mutant thyroid hormone alpha1 receptors (TR alpha1) were isolated from hepatocellular carcinomas of two patients. Sequence analyses of the complementary DNAs showed a single Val390Ala and double Pro398Ser/Glu350Lys mutations in mutants H and L, respectively. We characterized their hormone-binding, DNA-binding, and dominant negative activities. Mutants H and L did not ...

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Cell type-dependent modulation of the dominant negative action of human mutant thyroid hormone beta 1 receptors.

BACKGROUND Mutations in the ligand-binding domain of the thyroid hormone receptor beta (TR beta) gene cause the syndrome of resistance to thyroid hormone (RTH). The clinical phenotype results from the antagonism of the normal TR alpha and the non-mutated TR beta alleles by the TR beta 1 mutants, via a dominant negative effect. There is, however, marked heterogeneity of organ resistance within a...

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Biological activities of thyroid hormone receptors.

In recent years a large amount of data has been gathered on the biology of thyroid hormone receptors. The receptor proteins have been analyzed from the physiological and structural points of view. Most of the information has been summarized in several recent excellent reviews (1–6). The thyroid hormone receptors (TR) are ligand-modulated transcription factors which are members of a large superf...

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Embryonic lethal effect of expressing a dominant negative mutant human thyroid hormone receptor alpha1 in mice.

Resistance to thyroid hormone (RTH) is caused mainly by mutations of the thyroid hormone receptor (TR) beta gene. Although, in vitro, TRalpha1 and TRbeta1 mutants exhibit similar dominant negative effects against wild-type TR, no TRalpha mutants have ever been identified in RTH patients. It has been postulated that mutations in TRalpha gene may be lethal, compensated completely by intact TRbeta...

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ژورنال

عنوان ژورنال: Journal of Endocrinology

سال: 2000

ISSN: 0022-0795,1479-6805

DOI: 10.1677/joe.0.1670493