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| 1 | Novel ATP8B1 mutation in an adult male with progressive familial intrahepatic cholestasis显示文摘Progressive familial intrahepatic cholestasis type 1 is a rare disease that is characterized by low serum γ-glutamyltransferase levels due to mutation inATP8B1.We present a 23-year-old male who experienced persistent marked pruritus for eighteen years and recurrent jaundice for thirteen years,in addition to cholestasis that eventually became fatal.Genetic sequencing studies of the entire coding(exon) sequences of ATP8B1 and ABCB11 uncovered a novel heterozygous missense 3035G>T mutation(S1012I) and a synonymous 696T>C mutation in ATP8B1.The patient's progression was associated with not only impaired familial intrahepatic cholestasis 1(FIC1) function but also impaired bile salt export pump expression due to the impaired FIC1 function.Our findings show that patients with intermittent cholestasis can develop progressive liver disease even after several decades and require regular follow up. | Bao-Cheng Deng Sa Lv Wei Cui Rui Zhao Xu LU Jian Wu Pei Liu | 2012 | World Journal of Gastroenterology2012，18，44： | 2 |
| 2 | PECAM-1 is required for transendothelial migration of leukocytes 显示文摘 | Muller WA Weigl SA Deng XH | 1993 | J Exp Med1993，178，2： | 1 |
| 3 | Shifts in macrophage phe-notypes and macrophage competition for arginine metabolism affect the severity of muscle pathology in muscular dystrophy显示文摘 | Villalta SA Nguyen HX Deng B | 2009 | Hum Mol Genet2009，18，3： | 1 |
| 4 | Use of recombinant human bone morphogenetic protein-2 to enhance healing in a bone tunnel显示文摘 | Roder SA Suzuki K Deng XH | 1999 | Am J Sports Med1999，27，： | 1 |
| 5 | Shifts in macrophage phenotypes and macrophage competition for arginine metabo-lism affect the severity of muscle pathology in muscular dystro-phy显示文摘 | Villalta SA Nguyenl HX Deng B | | 0，，03： | 1 |
| 6 | Interleukin-10 reduces the pathology of mdx muscular dystrophy by deactivating M1 macrophages and modulating macrophage phenotype显示文摘 | Villalta SA Rinaldi C Deng B | | 0，，04： | 1 |
| 7 | Identification of a novel mutation of SH3BP2 in cherubism and demonstration that SH3BP2 mutations lead to increased NFAT activation显示文摘 | Lietman SA Kalinchinko N Deng X | 2006 | Hum Mutat2006，27，7： | 1 |
| 8 | PECAM-1 is required for trasendothelial migration of leukocytes显示文摘 | Muller WA Weigl SA Deng X | 1993 | J Exp Med1993，178，： | 1 |
| 9 | Use of recombinant human bone morphogenetic protein-2 to enhance tendon healing in a bone tunnel 显示文摘 | Rodeo SA Suzuki K Deng XH | 1999 | Am J Sports Med1999，27，： | 1 |
| 10 | IL-10 trig- gers changes in macrophage phenotype that promote mus- cle growth and regeneration显示文摘 | Deng B Wehling-Henricks M Villalta SA | 2012 | J Immunol2012，189，7： | 1 |
| 11 | Gene expression during development of fetal and adult Leydig cells显示文摘 | Deng L Jelinsky SA Finger JN | 2007 | Ann N Y Acad Sci2007，11，20： | 1 |
| 12 | Immunological response of female macaques to the PH-20 sperm protein following injection of recombinant proteins or synthesized peptides 显示文摘 | Deng X Meyers SA Tollner TL | 2002 | J Reprod Immu- nol2002，54，12： | 1 |
| 13 | A NIR fluorescent probe for Vanin-1 and its applications in imaging, kidney injury diagnosis,and the development of inhibitor显示文摘Vanin-1 is an amidohydrolase that catalyses the conversion of pantetheine into the aminothiol cysteamine and pantothenic acid(coenzyme A precursor), which plays a vital role in multiple physiological and pathological processes. In this study, an enzyme-activated near-infrared(NIR) fluorescent probe(DDAV) has been constructed for sensitively detecting Vanin-1 activity in complicated biosamples on the basis of its catalytic characteristics. DDAV exhibited a high selectivity and sensitivity toward Vanin-1 and was successfully applied to the early diagnosis of kidney injury in cisplatin-induced kidney injury model. In addition, DDAV could serve as a visual tool for in situ imaging endogenous Vanin-1 in vivo. More importantly, Enterococcus faecalis 20247 which possessed high expression of Vanin-1 was screened out from intestinal bacteria using DDAV, provided useful guidance for the rational use of NSAIDs in clinic. Finally, oleuropein as a potent natural inhibitor for Vanin-1 was discovered fromherbal medicines library using a high-throughput screening method using DDAV, which held great promise for clinical therapy of inflammatory bowel disease. | Zhenhao Tian Fei Yan Xiangge Tian Lei Feng Jingnan Cui Sa Deng Baojing Zhang Tian Xie Shanshan Huang Xiaochi Ma | 2022 | Acta Pharmaceutica Sinica B2022，12，1： | 1 |
| 14 | PECAM-1 is required for transendothelial migration of leukocytes显示文摘 | MullerWA Wuigl SA Deng X | 1993 | EXP Med1993，178，2： | 1 |
| 15 | Crossregulation of NF kappaB by the APC/GSK-3β/β-catenin pathway显示文摘 | Deng J Xia W Miller SA | 2004 | Mol Carcinog2004，3，3： | 1 |
| 16 | Beta-catenin interacts with and inhibits NF-kappa B in human colon and breast cancer显示文摘 | Deng J Miller SA Wang HY | 2002 | Camcer Cell2002，2，4： | 1 |
| 17 | Use of recombinant human bone morphogenetie protein-2 to enhance tendon healing in a bone tunnel显示文摘 | Rodeo SA Suzuki K Deng XH | 1999 | Am J Sports Meal1999，27，4： | 1 |
| 18 | Use of recombinant human bone morphogenetic protein- 2 to enhance tendon healing in a bone tunnel显示文摘 | Rodeo SA Suzuki K Deng XH | 1999 | Am J Sports Med1999，27，4： | 1 |
| 19 | Insulin Independence Following Isolated Islet Transplantation and Single Islet Infusions 显示文摘 | Markmann JF Deng SA Huang X | 2003 | Annals of Surgery2003，237，： | 1 |
| 20 | IL-10 trig-gers changes in macrophage phenotype that promote muscle growth and regeneration显示文摘 | Deng B Wehling-Henricks M Villalta SA | | 0，，07： | 1 |
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