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Evan M. de Joya Brett M. Colbert Pei-Ciao Tang Byron L. Lam Jun Yang Susan H. Blanton Derek M. Dykxhoorn Xuezhong Liu 《International journal of molecular sciences》2021,22(8)
Hearing loss is the most common sensory disorder with ~466 million people worldwide affected, representing about 5% of the population. A substantial portion of hearing loss is genetic. Hearing loss can either be non-syndromic, if hearing loss is the only clinical manifestation, or syndromic, if the hearing loss is accompanied by a collage of other clinical manifestations. Usher syndrome is a syndromic form of genetic hearing loss that is accompanied by impaired vision associated with retinitis pigmentosa and, in many cases, vestibular dysfunction. It is the most common cause of deaf-blindness. Currently cochlear implantation or hearing aids are the only treatments for Usher-related hearing loss. However, gene therapy has shown promise in treating Usher-related retinitis pigmentosa. Here we review how the etiologies of Usher-related hearing loss make it a good candidate for gene therapy and discuss how various forms of gene therapy could be applied to Usher-related hearing loss. 相似文献
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Meta-analysis was used to aggregate results from studies examining the relationship between intelligence and leadership. One hundred fifty-one independent samples in 96 sources met the criteria for inclusion in the meta-analysis. Results indicated that the corrected correlation between intelligence and leadership is .21 (uncorrected for range restriction) and .27 (corrected for range restriction). Perceptual measures of intelligence showed stronger correlations with leadership than did paper-and-pencil measures of intelligence. Intelligence correlated equally well with objective and perceptual measures of leadership. Additionally, the leader's stress level and the leader's directiveness moderated the intelligence-leadership relationship. Overall, results suggest that the relationship between intelligence and leadership is considerably lower than previously thought. The results also provide meta-analytic support for both implicit leadership theory and cognitive resource theory. (PsycINFO Database Record (c) 2010 APA, all rights reserved) 相似文献
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The RNA polymerase III factor TFIIIB forms a stable complex with DNA and can promote multiple rounds of initiation by polymerase. TFIIIB is composed of three subunits, the TATA binding protein (TBP), TFIIB-related factor (BRF), and B". Chemical footprinting, as well as mutagenesis of TBP, BRF, and promoter DNA, was used to probe the architecture of TFIIIB subunits bound to DNA. BRF bound to TBP-DNA through the nonconserved C-terminal region and required 15 bp downstream of the TATA box and as little as 1 bp upstream of the TATA box for stable complex formation. In contrast, formation of complete TFIIIB complexes required 15 bp both upstream and downstream of the TATA box. Hydroxyl radical footprinting of TFIIIB complexes and modeling the results to the TBP-DNA structure suggest that BRF and B" surround TBP on both faces of the TBP-DNA complex and provide an explanation for the exceptional stability of this complex. Competition for binding to TBP by BRF and either TFIIB or TFIIA suggests that BRF binds on the opposite face of the TBP-DNA complex from TFIIB and that the binding sites for TFIIA and BRF overlap. The positions of TBP mutations which are defective in binding BRF suggest that BRF binds to the top and N-terminal leg of TBP. One mutation on the N-terminal leg of TBP specifically affects the binding of the B" subunit. 相似文献
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Although cyclic nucleotide-gated channels mediate sensory transduction in olfaction and vision, other forms of sensory transduction are independent of these channels. Caenorhabditis elegans cyclic nucleotide-gated channel mutants respond normally to some olfactory stimuli and to osmotic stimuli, suggesting that these chemosensory responses use an alternative sensory transduction pathway. One gene that may act in this pathway is osm-9, which is required for each of these responses as well as a mechanosensory response to nose touch. osm-9 encodes a protein with ankyrin repeats and multiple predicted transmembrane domains that has limited similarity to the Drosophila phototransduction channels transient receptor potential (TRP) and TRP-like (TRPL). The sequence of OSM-9 and other TRP-like genes reveals a previously unsuspected diversity of mammalian and invertebrate genes in this family. osm-9 is required for the activity of the predicted G-protein-coupled odorant receptor ODR-10, which acts in the AWA olfactory neurons; its similarity to other G-protein-regulated transduction channels suggests that OSM-9 is involved in AWA signaling. osm-9:: GFP fusion genes are expressed in a subset of chemosensory, mechanosensory, and osmosensory neurons. osm-9 also affects olfactory adaptation within neurons that require the cyclic nucleotide-gated channel for olfaction; in these neurons, the gene has a regulatory function and not a primary role in sensory transduction. 相似文献
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Hollis Karen L.; Cadieux Elizabeth L.; Colbert M. Maura 《Canadian Metallurgical Quarterly》1989,103(2):115
Reproductive behavior can be elicited by signals that animals learn to associate with mates during repeated sexual encounters. Although researchers frequently have speculated about the evolutionary function of this Pavlovian-conditioned behavior, experimental evidence has been sparse. An experiment with blue gourami fish (Trichogaster trichopterus) demonstrates that both males and females learn to anticipate mates and that signaling enables territorial males to inhibit their characteristic initial aggressive response to females. Pavlovian-conditioned males exhibited more courtship appeasement behavior and less aggression than males whose contact with females was unsignaled. These data suggest that in nature Pavlovian conditioning can play an important role in the reproductive behavior of territorial species. (PsycINFO Database Record (c) 2010 APA, all rights reserved) 相似文献
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