An unliganded thyroid hormone receptor causes severe neurological dysfunction
نویسندگان
چکیده
منابع مشابه
Unliganded thyroid hormone receptor function: amphibian metamorphosis got TALENs.
Thyroid hormones (THs) are capital for development and cell homeostasis. THs act by regulating gene expression through TH receptors (TRs), which are transcription factors that belong to the nuclear receptors superfamily (1). THs and TRs are versatile players because they can not only up-regulate, but also down-regulate, gene expression in target cells. It is generally believed that TRs mediate ...
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Thyroid hormone, via its nuclear receptors TRalpha and TRbeta, controls metabolism by acting locally in peripheral tissues and centrally by regulating sympathetic signaling. We have defined aporeceptor regulation of metabolism by using mice heterozygous for a mutant TRalpha1 with low affinity to T3. The animals were hypermetabolic, showing strongly reduced fat depots, hyperphagia and resistance...
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Thyroid hormone (TH) is required for the development of vertebrates and exerts numerous homeostatic functions in adults. TH acts through nuclear receptors which control the transcription of target genes. Unliganded and liganded thyroid hormone receptors (TRs) have been shown to exert opposite effects on the transcription of target genes in vitro. However, the occurance of an aporeceptor activit...
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A new genetic disorder has been identified that results from mutation of THRA, encoding thyroid hormone receptor α1 (TRα1). Affected children have a high serum T3:T4 ratio and variable degrees of intellectual deficit and constipation but exhibit a consistently severe skeletal dysplasia. In an attempt to improve developmental delay and alleviate symptoms of hypothyroidism, patients are receiving...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2001
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.051454698