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Lee, Hee Keun,Song, Mee Hyun,Kang, Myengmo,Lee, Jung Tae,Kong, Kyoung-Ah,Choi, Su-Jin,Lee, Kyu Yup,Venselaar, Hanka,Vriend, Gert,Lee, Won-Sang,Park, Hong-Joon,Kwon, Taeg Kyu,Bok, Jinwoong,Kim, Un-Kyun American Physiological Society 2009 PHYSIOLOGICAL GENOMICS Vol.39 No.3
<P>X-linked deafness type 3 (DFN3), the most prevalent X-linked form of hereditary deafness, is caused by mutations in the POU3F4 locus, which encodes a member of the POU family of transcription factors. Despite numerous reports on clinical evaluations and genetic analyses describing novel POU3F4 mutations, little is known about how such mutations affect normal functions of the POU3F4 protein and cause inner ear malformations and deafness. Here we describe three novel mutations of the POU3F4 gene and their clinical characterizations in three Korean families carrying deafness segregating at the DFN3 locus. The three mutations cause a substitution (p.Arg329Pro) or a deletion (p.Ser310del) of highly conserved amino acid residues in the POU homeodomain or a truncation that eliminates both DNA-binding domains (p.Ala116fs). In an attempt to better understand the molecular mechanisms underlying their inner ear defects, we examined the behavior of the normal and mutant forms of the POU3F4 protein in C3H/10T1/2 mesodermal cells. Protein modeling as well as in vitro assays demonstrated that these mutations are detrimental to the tertiary structure of the POU3F4 protein and severely affect its ability to bind DNA. All three mutated POU3F4 proteins failed to transactivate expression of a reporter gene. In addition, all three failed to inhibit the transcriptional activity of wild-type proteins when both wild-type and mutant proteins were coexpressed. Since most of the mutations reported for DFN3 thus far are associated with regions that encode the DNA binding domains of POU3F4, our results strongly suggest that the deafness in DFN3 patients is largely due to the null function of POU3F4.</P>
Kwon, Tae-Jun,Cho, Hyun-Ju,Kim, Un-Kyung,Lee, Eujin,Oh, Se-Kyung,Bok, Jinwoong,Bae, Yong Chul,Yi, Jun-Koo,Lee, Jang Woo,Ryoo, Zae-Young,Lee, Sang Heun,Lee, Kyu-Yup,Kim, Hwa-Young IRL Press 2014 Human molecular genetics Vol.23 No.6
<P>Methionine sulfoxide reductase B3 (MsrB3) is a protein repair enzyme that specifically reduces methionine-<I>R</I>-sulfoxide to methionine. A recent genetic study showed that the <I>MSRB3</I> gene is associated with autosomal recessive hearing loss in human deafness DFNB74. However, the precise role of MSRB3 in the auditory system and the pathogenesis of hearing loss have not yet been determined. This work is the first to generate <I>MsrB3</I> knockout mice to elucidate the possible pathological mechanisms of hearing loss observed in DFNB74 patients. We found that homozygous <I>MsrB3<SUP>−/−</SUP></I> mice were profoundly deaf and had largely unaffected vestibular function, whereas heterozygous <I>MsrB3<SUP>+/−</SUP></I> mice exhibited normal hearing similar to that of wild-type mice. The MsrB3 protein is expressed in the sensory epithelia of the cochlear and vestibular tissues, beginning at E15.5 and E13.5, respectively. Interestingly, MsrB3 is densely localized at the base of stereocilia on the apical surface of auditory hair cells. MsrB3 deficiency led to progressive degeneration of stereociliary bundles starting at P8, followed by a loss of hair cells, resulting in profound deafness in <I>MsrB3<SUP>−/−</SUP></I> mice. The hair cell loss appeared to be mediated by apoptotic cell death, which was measured using TUNEL and caspase 3 immunocytochemistry. Taken together, our data suggest that MsrB3 plays an essential role in maintaining the integrity of hair cells, possibly explaining the pathogenesis of DFNB74 deafness in humans caused by MSRB3 deficiency.</P>
Lee, Sang-Ho,Choi, Kyung-Hwa,Cha, Kyu-Min,Hwang, Seock-Yeon,Park, Un-Kyu,Jeong, Min-Sik,Hong, Jae-Yup,Han, Chang-Kyun,In, Gyo,Kopalli, Spandana Rajendra,Kim, Si-Kwan The Korean Society of Ginseng 2019 Journal of Ginseng Research Vol.43 No.1
Background: Excessive stress causes varied physiological and psychological disorders including male reproductive problems. Here, we attempted to investigate the protective effects of Korean Red Ginseng (Panax ginseng Meyer; KRG) against sub-acute immobilization stress-induced testicular damage in experimental rats. Methods: Male rats (age, 4 wk; weight, 60-70 g) were divided into four groups (n = 8 in each group): normal control group, immobilization control group, immobilization group treated with 100 mg/kg of KRG daily, and immobilization group treated with 200 mg/kg of KRG daily. Normal control and immobilization control groups received vehicle only. KRG (100 mg/kg and 200 mg/kg) was mixed in the standard diet powder and fed daily for 6 mo. Parameters such as organ weight, blood chemistry, sperm kinematic values, and expression levels of testicular-related molecules were measured using commercially available kits, Western blotting, and reverse transcription polymerase chain reaction. Results: Data revealed that KRG restored the altered testis and epididymis weight in immobilization stress-induced rats significantly (p < 0.05). Further, KRG ameliorated the altered blood chemistry and sperm kinematic values when compared with the immobilization control group and attenuated the altered expression levels of spermatogenesis-related proteins (nectin-2, cAMP responsive element binding protein 1, and inhibin-${\alpha}$), sex hormone receptors (androgen receptor, luteinizing hormone receptor, and follicle-stimulating hormone receptor), and antioxidant-related enzymes (glutathione S-transferase m5, peroxiredoxin-4, and glutathione peroxidase 4) significantly in the testes of immobilization stress-induced rats. Conclusion: KRG protected immobilization stress-induced testicular damage and fertility factors in rats, thereby indicating its potential in the treatment of stress-related male sterility.
Facile Synthesis of Dendritic-Linear-Dendritic Materials by Click Chemistry
Lee, Jae-Wook,Han, Seung-Choul,Kim, Byoung-Ki,Lee, Un-Yup,Sung, Sae-Reum,Kang, Hwa-Shin,Kim, Ji-Hyeon,Jin, Sung-Ho The Polymer Society of Korea 2009 Macromolecular Research Vol.17 No.7
General, fast, and efficient stitching methods are presented for the synthesis of Fr$\acute{e}$chet-type dendrimers with linear units at a core, as a preliminary investigation for the synthesis of dendritic-linear-dendritic materials. The synthetic strategy involved an inexpensive, 1,3-dipolar, cycloaddition reaction between an alkyne and an azide in the presence of the Cu(I) species, which is known as the best example of click chemistry. The linear core building blocks, 1,7-octadiyne and 1,6-diazidohexane, were chosen to serve as the alkyne and azide functionalities for dendrimer growth via click reactions with the azide and alkyne-dendrons, respectively. These two building blocks were employed together with the azide- and alkyne-functionalized Fr$\acute{e}$chet-type dendrons in a convergent strategy to synthesize two kinds of Fr$\acute{e}$chet-type dendrimers with different linear core units. This comparative efficiency of the click methodology supports the fast and efficient synthesis of dendritic-linear-dendritic materials with the tailor made core unit.