Fam83h is Associated with Intracellular Vesicles and ADHCAI

Ding, Y.,Estrella, M.R.P.,Hu, Y.Y.,Chan, H.L.,Zhang, H.D.,Kim, J.-W.,Simmer, J.P.,Hu, J.C.-C. SAGE Publications 2009 Journal of dental research Vol.88 No.11

<P>Defects in <I>FAM83H</I> on human chromosome 8q24.3 cause autosomal-dominant hypocalcified amelogenesis imperfecta (ADHCAI). <I>FAM83H</I> does not encode a recognizable signal peptide, so we predicted that the Fam83h protein functions within the cell. We tested this hypothesis by constitutively expressing mouse Fam83h with green fluorescent protein (GFP) fused to its C-terminus in HEK293 and HeLa cell lines. Green fluorescent signal from the Fam83h-GFP fusion protein was associated with perinuclear vesicles, usually in the vicinity of the Golgi apparatus. No signal was observed within the nucleus. In addition, we identified <I> FAM83H</I> nonsense mutations in Hispanic (C1330C>T; p.Q444X) and Caucasian (c.1192C>T; p.Q398X) families with ADHCAI. We conclude that Fam83h localizes in the intracellular environment, is associated with vesicles, and plays an important role in dental enamel formation. <I>FAM83H</I> is the first gene involved in the etiology of amelogenesis imperfecta (AI) that does not encode a secreted protein.</P>

FAM83H mutations cause ADHCAI and alter intracellular protein localization.

Lee, S-K,Lee, K-E,Jeong, T-S,Hwang, Y-H,Kim, S,Hu, J C-C,Simmer, J P,Kim, J-W Journal of Dental Research, Inc 2011 Journal of dental research Vol.90 No.3

<P>Mutations in a family with sequence similarity 83 member H (FAM83H) cause autosomal-dominant hypocalcification amelogenesis imperfecta (ADH CAI). All FAM83H ADHCAI-causing mutations terminate translation or shift the reading frame within the specific exon 5 segment that encodes from Ser(287) to Glu(694). Mutations near Glu(694) cause a milder, more localized phenotype. We identified disease-causing FAM83H mutations in two families with ADHCAI: family 1 (g.3115C>T, c.1993 C>T, p.Q665X) and family 2 (g.3151C>T, c.2029 C>T, p.Q677X). We also tested the hypothesis that truncation mutations alter the intracellular localization of FAM83H. Wild-type FAM83H and p.E694X mutant FAM83H fused to green fluorescent protein (GFP) localized in the cytoplasm of HEK293T cells, but the mutant FAM83H proteins (p.R325X, p.W460X, and p.Q677X) fused to GFP localized mainly in the nucleus with slight expression in the cytoplasm. We conclude that nuclear targeting of the truncated FAM83H protein contributes to the severe, generalized enamel phenotype.</P>

Enhanced anti-tumor efficacy and safety profile of tumor microenvironment-responsive oncolytic adenovirus nanocomplex by systemic administration

Choi, J.W.,Dayananda, K.,Jung, S.J.,Lee, S.H.,Kim, D.,Hu, J.,Bae, Y.H.,Yun, C.O. Elsevier BV 2015 ACTA BIOMATERIALIA Vol.28 No.-

Oncolytic adenovirus (Ad) holds great promise as a potential gene therapy for cancer. However, intravenously administered Ad may encounter difficulties due to unfavorable host responses, non-specific interactions, and the heterogeneity of the tumor cell population. As an approach to combine the advantages of oncolytic Ad and synthetic polymers and to address the associated difficulties, Ad was physically complexed with a pH-sensitive block copolymer, methoxy poly(ethylene glycol)-b-poly(l-histidine) (mPEG-b-pHis). The in vitro transduction efficiency at an acidic extracellular pH was remarkably enhanced in cancer cells when treated with the Ad expressing green fluorescent protein (GFP) coated with mPEG-b-pHis (c-dE1/GFP) as compared to that of naked Ad (n-dE1/GFP). Time-lapse total internal reflection fluorescence microscopic imaging revealed a significantly enhanced cellular uptake rate of c-dE1/GFP at acidic tumor pH when compared with that at neutral pH or naked cognate Ad (n-dE1/GFP). In addition, c-dE1/GFP remained relatively stable in human serum-containing media, and considerably reduced both the innate and adaptive immune response against Ad. Moreover, the therapeutic efficacy and survival benefit of mPEG-b-pHis-complexed oncolytic Ad (c-H5mT/Luc) by systemic treatment was significantly enhanced compared to that with naked oncolytic Ad (n-H5mT/Luc) in both coxsackie and adenovirus receptor-positive and -negative tumors. Whole-body bioluminescence imaging showed 7.3-fold higher luciferase expression at the tumor site and 23.0-fold less luciferase expression in liver tissue for c-H5mT/Luc relative to that for naked oncolytic Ad (n-H5mT/Luc). Considering the heterogeneity of tumor tissue, these results are important for guiding the development of more potent and specific treatment of devastating metastatic cancers using this viral system. Statement of significance: Although adenoviral systems have shown considerable promise and undergone extensive evaluation attempts to specifically target Ad vectors to cancer cells have met limited success. This shortcoming is due to the strong immune response stimulated by Ad and the hepatotoxicity of the viral particles. To overcome restricted vector issues, we generated Ad/mPEG-b-pHis for tumor microenvironment-targeting hybrid vector systems, an oncolytic Ad coated with a pH-responsive polymer, mPEG-b-pHis. The Ad/mPEG-b-pHis exhibited pH-dependent transduction efficiency and cancer-cell killing effects. Moreover, systemic administration of oncolytic Ad/mPEG-b-pHis led to marked suppression of tumor growth and tumor-specific viral replication. Ad successfully avoided the innate and adaptive immune responses and liver accumulation with the help of mPEG-b-pHis on its surface.

Synthesis, Structure and Magnetization Behaviors of MnBi/Fe₃B/Nd₂Fe<SUB>14</SUB>B Nanocomposite alloy

Y. Yang,Q. Wu,Y. C. Hu,P. Y. Zhang,H. L. Ge 한국자기학회 2016 Journal of Magnetics Vol.21 No.2

Microstructure and magnetization behaviors of MnBi/Fe₃B/Nd₂Fe14B nanocomposite alloy have been investigated. It was found that the coercivity increased firstly and then decreased, and saturation magnetization decreased with the additon of MnBi alloy. The addition of 40 wt.% MnBi powder enhanced the coercivity from 192.8 kA/m to 311.2 kA/m. The δM and D(H)-H plots suggested the occurrence of a stronger exchange-coupling occurring between the hard and soft magnetic phase for this sample. The dependence of coercivity with temperature was discussed in 40 wt.% Mn55Bi45/ 60 wt.% Nd4.5Fe76.5Nb0.5B18.5 alloy powder, and a positive temperature coefficient was founded from 298 K to 350 K.

A Comparison Study between Convex-Louver , Wavy , and Plain Fin-and-Tube Heat Exchangers

C. C. Wang,Y. Z. Robert Hu,Y. M. Tsai 대한기계학회 1998 Heat transter conference Vol.6 No.1

Phospholipase C delta 1 is a novel 3p22.3 tumor suppressor involved in cytoskeleton organization, with its epigenetic silencing correlated with high-stage gastric cancer

Hu, X -T,Zhang, F -B,Fan, Y -C,Shu, X -S,Wong, A H Y,Zhou, W,Shi, Q -L,Tang, H -M,Fu, L,Guan, X -Y,Rha, S Y,Tao, Q,He, C Macmillan Publishers Limited 2009 Oncogene Vol.28 No.26

Located at the important tumor suppressor locus, 3p22, PLCD1 encodes an enzyme that mediates regulatory signaling of energy metabolism, calcium homeostasis and intracellular movements. We identified PLCD1 as a downregulated gene in aerodigestive carcinomas through expression profiling and epigenetic characterization. We found that PLCD1 was expressed in all normal adult tissues but low or silenced in 84% (16/19) gastric cancer cell lines, well correlated with its CpG island (CGI) methylation status. Methylation was further detected in 62% (61/98) gastric primary tumors, but none of normal gastric mucosa tissues. PLCD1 methylation was significantly correlated with tumor high stage. Detailed methylation analysis of 37 CpG sites at the PLCD1 CGI by bisulfite genomic sequencing confirmed its methylation. PLCD1 silencing could be reversed by pharmacological demethylation with 5-aza-2′-deoxycytidine, indicating a direct epigenetic silencing. Ectopic expression of PLCD1 in silenced gastric tumor cells dramatically inhibited their clonogenicity and migration, possibly through downregulating MMP7 expression and hampering the reorganization of cytoskeleton through cofilin inactivation by phosphorylation. Thus, epigenetic inactivation of PLCD1 is common and tumor-specific in gastric cancer, and PLCD1 acts as a functional tumor suppressor involved in gastric carcinogenesis.Oncogene (2009) 28, 2466–2475; doi:10.1038/onc.2009.92; published online 18 May 2009

DISCOVERY OF AN X-RAY-EMITTING CONTACT BINARY SYSTEM 2MASS J11201034−2201340

Hu, Chin-Ping,Yang, Ting-Chang,Chou, Yi,Liu, L.,Qian, S.-B.,Hui, C. Y.,Kong, Albert K. H.,Lin, L. C. C.,Tam, P. H. T.,Li, K. L.,Ngeow, Chow-Choong,Chen, W. P.,Ip, Wing-Huen American Astronomical Society 2016 The Astronomical journal Vol.151 No.6

<P>We report the detection of orbital modulation, a model solution, and the X-ray properties of a newly discovered contact binary, Two Micron All Sky Survey (2MASS) J11201034-2201340. We serendipitously found this X-ray point source outside the error ellipse when searching for possible X-ray counterparts of 7-ray millisecond pulsars among the unidentified objects detected by the Fermi Gamma-ray Space Telescope. The optical counterpart of the X-ray source (unrelated to the 7-ray source) was then identified using archival databases. The long-term Catalina Real-Time Transient Survey detected a precise signal with a period of P = 0.28876208 (56) days. A follow-up observation made by the Super Light Telescope of Lulin Observatory revealed the binary nature of the object. Utilizing archived photometric data of multi-band surveys, we construct the spectral energy distribution (SED), which is well fit by a K2V spectral template. The fitting result of the orbital profile using the Wilson Devinney code suggests that 2MASS J11201034-2201340 is a short-period A-type contact binary and the more massive component has a cool spot. The X-ray emission was first noted in observations made by Swift, and then further confirmed and characterized by an XMM-Newton observation. The X-ray spectrum can be described by a power law or thermal Bremsstrahlung. Unfortunately, we could not observe significant X-ray orbital modulation. Finally, according to the SED, this system is estimated to be 690 pc from Earth with a calculated X-ray intensity of (0.7 - 1.5) x 10(30) erg s(-1), which is in the expected range of an X-ray emitting contact binary.</P>

Multi-pass laser welding of thick plate with filler wire by using a narrow gap joint configuration

Y. C. Yu,S. L. Yang,Y. Yin,C. M. Wang,X. Y. Hu,X. X. Meng,S. F. Yu 대한기계학회 2013 JOURNAL OF MECHANICAL SCIENCE AND TECHNOLOGY Vol.27 No.7

A 17 mm low-carbon steel plate is welded by a fiber laser with a narrow gap joint configuration and multi-pass technique. A highspeed camera is used to real-time monitor the welding process, and the effects of groove size and the side shielding gas on the weld quality are studied. Experimental results showed that a concave shape in the bottom of weld can be formed when use a general shielding gas nozzle with the 8.0 mm external diameter. Through a special design of the shielding gas nozzle with the 2.0 mm external diameter which can deep into the interior of groove, the pressure from shielding gas can balance the surface tension from the liquid in the top of groove,so the shielding effect can be dramatically improved and the concave shape in the bottom of weld can be eliminated. When the filler wire and laser beam can smoothly enter the groove, using a relatively small groove size not only reduce the consumption of filler wire but also reduce the deflection of filler wire in the gap that can improve the fusion of groove.

DETECTION OF X-RAY PERIODICITY FROM A NEW ECLIPSING POLAR CANDIDATE XGPS-I J183251-100106

Hui, C. Y.,Seo, K. A.,Hu, C. P.,Lin, L. C. C.,Chou, Y. IOP Publishing 2012 The Astrophysical journal Vol.759 No.2

<P>We report the results from a detailed analysis of an archival XMM-Newton observation of the X-ray source XGPS-I J183251-100106, which has been suggested as a promising magnetic cataclysmic variable (CV) candidate based on its optical properties. A single periodic signal of similar to 1.5 is detected from all EPIC instruments on board XMM-Newton. The phase-averaged X-ray spectrum can be well modeled with a thermal bremsstrahlung temperature of kT similar to 50 keV. Both the X-ray spectral and temporal behavior of this system suggest that it is an eclipsing CV of the AM Herculis (or polar) type.</P>

SEARCHES FOR MILLISECOND PULSAR CANDIDATES AMONG THE UNIDENTIFIED<i>FERMI</i>OBJECTS

Hui, C. Y.,Park, S. M.,Hu, C. P.,Lin, L. C. C.,Li, K. L.,Kong, A. K. H.,Tam, P. H. T.,Takata, J.,Cheng, K. S.,Jin, Ruolan,Yen, T.-C.,Kim, Chunglee IOP Publishing 2015 The Astrophysical journal Vol.809 No.1

<P>Here we report the results of searching millisecond pulsar (MSP) candidates from the Fermi LAT second source catalog (2FGL). Seven unassociated gamma-ray sources in this catalog are identified as promising MSP candidates based on their gamma-ray properties. Through the X-ray analysis, we have detected possible X-ray counterparts, localized to an arcsecond accuracy. We have systematically estimated their X-ray fluxes and compared them with the corresponding gamma-ray fluxes. The X-ray to gamma-ray flux ratios for 2FGL J1653.6-0159 and 2FGL J1946.4-5402 are comparable with the typical value for pulsars. For 2FGL J1625.2-0020, 2FGL J1653.6-0159, and 2FGL J1946.4-5402, their candidate X-ray counterparts are bright enough to perform a detailed spectral and temporal analysis to discriminate their thermal/non-thermal nature and search for the periodic signal. We have also searched for possible optical/IR counterparts at the X-ray positions. For the optical/IR source coincident with the brightest X-ray object associated with 2FGL J1120.0-2204, its spectral energy distribution is comparable with a late-type star. Evidence for the variability has also been found by examining its optical light curve. All the aforementioned 2FGL sources resemble a pulsar in one or more aspects, making them promising targets for follow-up investigations.</P>