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Items: 44


Plectin Mutations in Progressive Familial Intrahepatic Cholestasis.

Wu SH, Hsu JS, Chen HL, Chien MM, Wu JF, Ni YH, Liou BY, Ho MC, Jeng YM, Chang MH, Chen PL, Chen HL.

Hepatology. 2019 Jul 3. doi: 10.1002/hep.30841. [Epub ahead of print]


Combination of acid β-glucosidase mutation and Saposin C deficiency in mice reveals Gba1 mutation dependent and tissue-specific disease phenotype.

Liou B, Zhang W, Fannin V, Quinn B, Ran H, Xu K, Setchell KDR, Witte D, Grabowski GA, Sun Y.

Sci Rep. 2019 Apr 3;9(1):5571. doi: 10.1038/s41598-019-41914-7.


Jaundice revisited: recent advances in the diagnosis and treatment of inherited cholestatic liver diseases.

Chen HL, Wu SH, Hsu SH, Liou BY, Chen HL, Chang MH.

J Biomed Sci. 2018 Oct 26;25(1):75. doi: 10.1186/s12929-018-0475-8. Review.


Panel-Based Next-Generation Sequencing for the Diagnosis of Cholestatic Genetic Liver Diseases: Clinical Utility and Challenges.

Chen HL, Li HY, Wu JF, Wu SH, Chen HL, Yang YH, Hsu YH, Liou BY, Chang MH, Ni YH.

J Pediatr. 2019 Feb;205:153-159.e6. doi: 10.1016/j.jpeds.2018.09.028. Epub 2018 Oct 23.


Use of Autometallography to Localize and Semi-Quantify Silver in Cetacean Tissues.

Li WT, Liou BY, Yang WC, Chen MH, Chang HW, Chiou HY, Pang VF, Jeng CR.

J Vis Exp. 2018 Oct 4;(140). doi: 10.3791/58232.


Sulbactam-enhanced cytotoxicity of doxorubicin in breast cancer cells.

Wen SH, Su SC, Liou BH, Lin CH, Lee KR.

Cancer Cell Int. 2018 Sep 4;18:128. doi: 10.1186/s12935-018-0625-9. eCollection 2018.


Less Severe but Prolonged Course of Acute Hepatitis A in Human Immunodeficiency Virus (HIV)-Infected Patients Compared With HIV-Uninfected Patients During an Outbreak: A Multicenter Observational Study.

Lee YL, Chen GJ, Chen NY, Liou BH, Wang NC, Lee YT, Yang CJ, Huang YS, Tang HJ, Huang SS, Lin YC, Cheng CY, Lee CH, Chen TC, Wu TS, Liu CE, Lu PL, Hung CC; Taiwan HIV Study Group .

Clin Infect Dis. 2018 Oct 30;67(10):1595-1602. doi: 10.1093/cid/ciy328.


Investigation of silver (Ag) deposition in tissues from stranded cetaceans by autometallography (AMG).

Li WT, Chang HW, Chen MH, Chiou HY, Liou BY, Pang VF, Yang WC, Jeng CR.

Environ Pollut. 2018 Apr;235:534-545. doi: 10.1016/j.envpol.2018.01.010. Epub 2018 Jan 9.


Associations of melatonin receptor gene polymorphisms with Graves' disease.

Lin JD, Yang SF, Wang YH, Fang WF, Lin YC, Liou BC, Lin YF, Tang KT, Cheng CW.

PLoS One. 2017 Sep 29;12(9):e0185529. doi: 10.1371/journal.pone.0185529. eCollection 2017.


The effect of manganese exposure in Atp13a2-deficient mice.

Fleming SM, Santiago NA, Mullin EJ, Pamphile S, Karkare S, Lemkuhl A, Ekhator OR, Linn SC, Holden JG, Aga DS, Roth JA, Liou B, Sun Y, Shull GE, Schultheis PJ.

Neurotoxicology. 2018 Jan;64:256-266. doi: 10.1016/j.neuro.2017.06.005. Epub 2017 Jun 6.


Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease.

Pandey MK, Burrow TA, Rani R, Martin LJ, Witte D, Setchell KD, Mckay MA, Magnusen AF, Zhang W, Liou B, Köhl J, Grabowski GA.

Nature. 2017 Mar 2;543(7643):108-112. doi: 10.1038/nature21368. Epub 2017 Feb 22.


Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice.

Liou B, Peng Y, Li R, Inskeep V, Zhang W, Quinn B, Dasgupta N, Blackwood R, Setchell KD, Fleming S, Grabowski GA, Marshall J, Sun Y.

Hum Mol Genet. 2016 Dec 1;25(23):5126-5141. doi: 10.1093/hmg/ddw322.


Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.

Dai M, Liou B, Swope B, Wang X, Zhang W, Inskeep V, Grabowski GA, Sun Y, Pan D.

PLoS One. 2016 Sep 6;11(9):e0162367. doi: 10.1371/journal.pone.0162367. eCollection 2016.


Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model.

Dasgupta N, Xu YH, Li R, Peng Y, Pandey MK, Tinch SL, Liou B, Inskeep V, Zhang W, Setchell KD, Keddache M, Grabowski GA, Sun Y.

Hum Mol Genet. 2015 Dec 15;24(24):7031-48. doi: 10.1093/hmg/ddv404. Epub 2015 Sep 29.


Efficacy of tigecycline for secondary Acinetobacter bacteremia and factors associated with treatment failure.

Liou BH, Lee YT, Kuo SC, Liu PY, Fung CP.

Antimicrob Agents Chemother. 2015;59(6):3637-40. doi: 10.1128/AAC.04987-14. Epub 2015 Mar 30.


Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology.

Sun Y, Florer J, Mayhew CN, Jia Z, Zhao Z, Xu K, Ran H, Liou B, Zhang W, Setchell KD, Gu J, Grabowski GA.

PLoS One. 2015 Mar 30;10(3):e0118771. doi: 10.1371/journal.pone.0118771. eCollection 2015.


Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.

Barnes S, Xu YH, Zhang W, Liou B, Setchell KD, Bao L, Grabowski GA, Sun Y.

PLoS One. 2014 Dec 31;9(12):e116023. doi: 10.1371/journal.pone.0116023. eCollection 2014.


Prenatal diagnosis of complete ring chromosome 22 without phenotypical abnormalities.

Liou B, Su CF, Luo CH, Tsai HJ.

Taiwan J Obstet Gynecol. 2014 Sep;53(3):406-8. doi: 10.1016/j.tjog.2013.06.016. No abstract available.


The LIMP-2/SCARB2 binding motif on acid β-glucosidase: basic and applied implications for Gaucher disease and associated neurodegenerative diseases.

Liou B, Haffey WD, Greis KD, Grabowski GA.

J Biol Chem. 2014 Oct 24;289(43):30063-74. doi: 10.1074/jbc.M114.593616. Epub 2014 Sep 8.


Risk factors and clinical outcome of sulbactam nonsusceptibility in monomicrobial Acinetobacter nosocomialis bacteremia.

Lai HH, Liou BH, Chang YY, Kuo SC, Lee YT, Chen TL, Fung CP.

J Microbiol Immunol Infect. 2016 Jun;49(3):371-7. doi: 10.1016/j.jmii.2014.06.004. Epub 2014 Jul 28.


Reversal of advanced disease in lysosomal acid lipase deficient mice: a model for lysosomal acid lipase deficiency disease.

Sun Y, Xu YH, Du H, Quinn B, Liou B, Stanton L, Inskeep V, Ran H, Jakubowitz P, Grilliot N, Grabowski GA.

Mol Genet Metab. 2014 Jul;112(3):229-41. doi: 10.1016/j.ymgme.2014.04.006. Epub 2014 May 4.


Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice.

Xu YH, Xu K, Sun Y, Liou B, Quinn B, Li RH, Xue L, Zhang W, Setchell KD, Witte D, Grabowski GA.

Hum Mol Genet. 2014 Aug 1;23(15):3943-57. doi: 10.1093/hmg/ddu105. Epub 2014 Mar 5.


Reduced efficiency droop in blue InGaN light-emitting diodes by thin AlGaN barriers.

Chang JY, Chang YA, Wang TH, Chen FM, Liou BT, Kuo YK.

Opt Lett. 2014 Feb 1;39(3):497-500. doi: 10.1364/OL.39.000497.


Dinuclear gold(I) dithio- and diselenophosph(in)ate complexes forming mononuclear gold(III) oxidative addition complexes and reversible chemical reductive elimination products.

Lee YC, Lin YR, Liou BY, Liao JH, Gusarova NK, Trofimov BA, van Zyl WE, Liu CW.

Dalton Trans. 2014 Jan 14;43(2):663-70. doi: 10.1039/c3dt52557k. Epub 2013 Oct 18.


A multicenter surveillance of antimicrobial resistance in Serratia marcescens in Taiwan.

Liou BH, Duh RW, Lin YT, Lauderdale TL, Fung CP; Taiwan Surveillance of Antimicrobial Resistance (TSAR) Hospitals.

J Microbiol Immunol Infect. 2014 Oct;47(5):387-93. doi: 10.1016/j.jmii.2013.04.003. Epub 2013 Jun 14.


Quantifying membrane permeability of amphotericin B ion channels in single living cells.

Yang TS, Ou KL, Peng PW, Liou BC, Wang WT, Huang YC, Tsai CM, Su CH.

Biochim Biophys Acta. 2013 Aug;1828(8):1794-801. doi: 10.1016/j.bbamem.2013.03.021. Epub 2013 Apr 2.


Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease.

Sun Y, Zhang W, Xu YH, Quinn B, Dasgupta N, Liou B, Setchell KD, Grabowski GA.

PLoS One. 2013;8(3):e57560. doi: 10.1371/journal.pone.0057560. Epub 2013 Mar 8.


SESN-1 is a positive regulator of lifespan in Caenorhabditis elegans.

Yang YL, Loh KS, Liou BY, Chu IH, Kuo CJ, Chen HD, Chen CS.

Exp Gerontol. 2013 Mar;48(3):371-9. doi: 10.1016/j.exger.2012.12.011. Epub 2013 Jan 11.


Is E326K glucocerebrosidase a polymorphic or pathological variant?

Liou B, Grabowski GA.

Mol Genet Metab. 2012 Mar;105(3):528-9. doi: 10.1016/j.ymgme.2011.12.002. Epub 2011 Dec 8. No abstract available.


Ex vivo and in vivo effects of isofagomine on acid β-glucosidase variants and substrate levels in Gaucher disease.

Sun Y, Liou B, Xu YH, Quinn B, Zhang W, Hamler R, Setchell KD, Grabowski GA.

J Biol Chem. 2012 Feb 3;287(6):4275-87. doi: 10.1074/jbc.M111.280016. Epub 2011 Dec 13.


Numerical investigation on the enhanced carrier collection efficiency of Ga-face GaN/InGaN p-i-n solar cells with polarization compensation interlayers.

Chang JY, Liou BT, Lin HW, Shih YH, Chang SH, Kuo YK.

Opt Lett. 2011 Sep 1;36(17):3500-2. doi: 10.1364/OL.36.003500.


Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse.

Sun Y, Ran H, Liou B, Quinn B, Zamzow M, Zhang W, Bielawski J, Kitatani K, Setchell KD, Hannun YA, Grabowski GA.

PLoS One. 2011 Apr 20;6(4):e19037. doi: 10.1371/journal.pone.0019037.


Secondary metabolites from the leaves of Neolitsea hiiranensis and the anti-inflammatory activity of some of them.

Liou BJ, Chang HS, Wang GJ, Chiang MY, Liao CH, Lin CH, Chen IS.

Phytochemistry. 2011 Apr;72(4-5):415-22. doi: 10.1016/j.phytochem.2011.01.006. Epub 2011 Feb 2.


A complicated hematometra and fallopian tubal occlusion in uterine didelphys with unilateral cervical atresia.

Su CF, Tsui KP, Liou B, Ruan CW, Tsai HJ.

Taiwan J Obstet Gynecol. 2010 Dec;49(4):515-7. doi: 10.1016/S1028-4559(10)60107-6. No abstract available.


Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits.

Sun Y, Liou B, Ran H, Skelton MR, Williams MT, Vorhees CV, Kitatani K, Hannun YA, Witte DP, Xu YH, Grabowski GA.

Hum Mol Genet. 2010 Mar 15;19(6):1088-97. doi: 10.1093/hmg/ddp580. Epub 2010 Jan 4.


In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models.

Sun Y, Liou B, Quinn B, Ran H, Xu YH, Grabowski GA.

PLoS One. 2009 Oct 7;4(10):e7320. doi: 10.1371/journal.pone.0007320.


Participation of asparagine 370 and glutamine 235 in the catalysis by acid beta-glucosidase: the enzyme deficient in Gaucher disease.

Liou B, Grabowski GA.

Mol Genet Metab. 2009 May;97(1):65-74. doi: 10.1016/j.ymgme.2009.01.006. Epub 2009 Feb 13.


Analyses of variant acid beta-glucosidases: effects of Gaucher disease mutations.

Liou B, Kazimierczuk A, Zhang M, Scott CR, Hegde RS, Grabowski GA.

J Biol Chem. 2006 Feb 17;281(7):4242-53. Epub 2005 Nov 17.


On the thermal annealing conditions for self-synthesis of tungsten carbide nanowires from WC(x) films.

Wang SJ, Chen CH, Chang SC, Wong CH, Uang KM, Chen TM, Ko RM, Liou BW.

Nanotechnology. 2005 Feb;16(2):273-7. doi: 10.1088/0957-4484/16/2/016. Epub 2005 Jan 13.


Scar endometriosis.

Liang CC, Liou B, Tsai CC, Chen TC, Soong YK.

Int Surg. 1998 Jan-Mar;83(1):69-71.


Gaucher disease: four families with previously undescribed mutations.

Beutler E, Gelbart T, Balicki D, Demina A, Adusumalli J, Elsas L 2nd, Grinzaid KA, Gitzelmann R, Superti-Furga A, Kattamis C, Liou BB.

Proc Assoc Am Physicians. 1996 May;108(3):179-84.


Gaucher disease: identification of three new mutations in the Korean and Chinese (Taiwanese) populations.

Kim JW, Liou BB, Lai MY, Ponce E, Grabowski GA.

Hum Mutat. 1996;7(3):214-8.


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