Benjamin Liou

Benjamin Liou

UNVERIFIED PROFILE

Are you Benjamin Liou?   Register this Author

Register author
Benjamin Liou

Benjamin Liou

Publications by authors named "Benjamin Liou"

Are you Benjamin Liou?   Register this Author

20Publications

614Reads

15Profile Views

The effect of manganese exposure in Atp13a2-deficient mice.

Neurotoxicology 2018 01 6;64:256-266. Epub 2017 Jun 6.

Department of Biological Sciences, Northern Kentucky University, Highland Heights, KY, United States.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neuro.2017.06.005DOI Listing
January 2018

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

PLoS One 2016 6;11(9):e0162367. Epub 2016 Sep 6.

Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States of America.

View Article

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0162367PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5012639PMC
August 2017

Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease.

Nature 2017 03 22;543(7643):108-112. Epub 2017 Feb 22.

Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1038/nature21368DOI Listing
March 2017

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

PLoS One 2015 30;10(3):e0118771. Epub 2015 Mar 30.

Division of Human Genetics, Cincinnati Children's Hospital Research Foundation, University of Cincinnati College of Medicine, Cincinnati, Ohio, United States of America; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, United States of America; Synageva BioPharma Corp., Lexington, Massachusetts, United States of America.

View Article

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0118771PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4378893PMC
February 2016

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

Hum Mol Genet 2015 Dec 29;24(24):7031-48. Epub 2015 Sep 29.

Division of Human Genetics, Cincinnati Children's Hospital Research Foundation, Cincinnati, OH 45229, USA, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/hmg/ddv404DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4654057PMC
December 2015

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

PLoS One 2014 31;9(12):e116023. Epub 2014 Dec 31.

The Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States of America; The Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, United States of America.

View Article

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0116023PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4281226PMC
October 2015

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

J Biol Chem 2014 Oct 8;289(43):30063-74. Epub 2014 Sep 8.

From the Division of Human Genetics, Cincinnati Children's Hospital Medical Center, and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229 and

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1074/jbc.M114.593616DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4208013PMC
October 2014

Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice.

Hum Mol Genet 2014 Aug 5;23(15):3943-57. Epub 2014 Mar 5.

The Division of Human Genetics and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA Synageva BioPharma, Lexington, MA 02421, USA

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/hmg/ddu105DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4082362PMC
August 2014

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

Mol Genet Metab 2014 Jul 4;112(3):229-41. Epub 2014 May 4.

The Division of Human Genetics, Cincinnati Children's Hospital Research Foundation, USA; The Department of Pediatrics, University of Cincinnati College of Medicine Cincinnati, OH 45229, USA; Synageva BioPharma Corp., Lexington, MA 02421, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ymgme.2014.04.006DOI Listing
July 2014

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

J Biol Chem 2012 Feb 13;287(6):4275-87. Epub 2011 Dec 13.

Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1074/jbc.M111.280016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3281716PMC
February 2012

Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse.

PLoS One 2011 Apr 20;6(4):e19037. Epub 2011 Apr 20.

The Division of Human Genetics, University of Cincinnati College of Medicine, Cincinnati, Ohio, United States of America.

View Article

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0019037PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3080394PMC
April 2011

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

Mol Genet Metab 2009 May 13;97(1):65-74. Epub 2009 Feb 13.

The A. Graeme Mitchell Chair in Human Genetics, Division and Program in Human Genetics, Children's Hospital Medical Center, 3333 Burnet Avenue, ML 4006, Cincinnati, OH 45229-3039, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ymgme.2009.01.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2699545PMC
May 2009

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

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

Division and Program in Human Genetics, Children's Hospital Research Foundation, Cincinnati, OH 45229, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1074/jbc.M511110200DOI Listing
February 2006