Anti-THRA (aa 246-295) polyclonal antibody

The deafness caused by early onset hypothyroidism indicates that thyroid hormone is essential for the development of hearing. We investigated the underlying roles of the TR alpha1 and TR beta thyroid hormone receptors in the auditory system using receptor-deficient mice. TR alpha1 and TR beta, which act as hormone-activated transcription factors, are encoded by the Thra and Thrb genes, respectively, and both are expressed in the developing cochlea. TR beta is required for hearing because TR beta -deficient (Thrb(tm1/tm1)) mice have a defective auditory-evoked brainstem response and retarded expression of a potassium current (I-K,I-f) in the cochlear inner hair cells. Here, we show that although TR alpha1 is individually dispensable, TR alpha1 and TR beta synergistically control an extended array of functions in postnatal cochlear development. Compared with Thrb(tm1/tm1) mice, the deletion of all TRs in Thra(tm1/tm1)Thrb(tm1/tm1) mice produces exacerbated and novel phenotypes, including delayed differentiation of the sensory epithelium, malformation of the tectorial membrane, impairment of electromechanical transduction in outer hair cells, and a low endocochlear potential. The induction of I-K,I-f in inner hair cells was not markedly more retarded than in Thrb(tm1/tm1) mice, suggesting that this feature of hair cell maturation is primarily TR beta -dependent. These results indicate that distinct pathways mediated by TR beta alone or by TR beta and TR alpha1 together facilitate control over an extended range of functions during the maturation of the cochlea.

Kawasaki disease (KD) is anacute febrile coronary vasculitis disease in children. In general, this disease can be treated with a single dose of 2 g/kg intravenous immunoglobulin (IVIG). However, the best timing for administering steroid treatment in acute-stage KD is still under debate. In this study, we recruited 174 participants to survey the transcript levels of steroid hormone receptors in KD patients. The chip studies consisted of 18 KD patients that were analyzed before IVIG treatment and at least 3 weeks after IVIG administration, as well as 36 control subjects, using GeneChip (R) HTA 2.0. Another cohort consisting of 120 subjects was analyzed to validate qRT-PCR. Our microarray study demonstrated significant downregulated expressions of the mRNA levels of NR1A2, RORA, NR4A1-3, THRA, and PPARD in KD patients in comparision to the controls. However, these genes increased considerably in KD patients after IVIG administration. After PCR validation, our data only revealed decreased NR4A2 mRNA expression in the KD patients compared to those of the controls, which increased after they received IVIG treatment. Our study is the first to report the potential effective utilization of steroid treatment in KD. Prior to IVIG treatment, decreased steroid receptors allowed for the reduced treatment role of steroids. However, after IVIG treatment, increased steroid receptors indicate that steroids are effective as a supplementary treatment for KD.