David A Wenger

David A Wenger

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David A Wenger

David A Wenger

Publications by authors named "David A Wenger"

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The Lysosomal Diseases Testing Laboratory: A review of the past 47 years.

JIMD Rep 2020 Jul 4;54(1):61-67. Epub 2020 Apr 4.

Department of Neurology Sidney Kimmel College of Medicine at Thomas Jefferson University Philadelphia Pennsylvania USA.

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http://dx.doi.org/10.1002/jmd2.12117DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7358674PMC
July 2020

Lysosomal storage disease spectrum in nonimmune hydrops fetalis: a retrospective case control study.

Prenat Diagn 2020 05 20;40(6):738-745. Epub 2020 Mar 20.

Lysosomal Diseases Testing Laboratory, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1002/pd.5678DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7260084PMC
May 2020

Conditions for combining gene therapy with bone marrow transplantation in murine Krabbe disease.

Bioimpacts 2020 24;10(2):105-115. Epub 2020 Mar 24.

Department of Neurology, Sidney Kimmel College of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.34172/bi.2020.13DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7186542PMC
March 2020

Rare Saposin A deficiency: Novel variant and psychosine analysis.

Mol Genet Metab 2020 02 5;129(2):161-164. Epub 2019 Aug 5.

Lucile Packard Children's Hospital Stanford, 725 Welch Road, Palo Alto, CA 94304, United States of America; Department of Pediatrics, Division of Medical Genetics, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, United States of America. Electronic address:

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http://dx.doi.org/10.1016/j.ymgme.2019.08.001DOI Listing
February 2020

Biochemical and clinical response after umbilical cord blood transplant in a boy with early childhood-onset beta-mannosidosis.

Mol Genet Genomic Med 2019 07 21;7(7):e00712. Epub 2019 May 21.

Division of Pediatric Blood and Marrow Transplant, University of Minnesota, Minneapolis, Minnesota.

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http://dx.doi.org/10.1002/mgg3.712DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625138PMC
July 2019

A closer look at ARSA activity in a patient with metachromatic leukodystrophy.

Mol Genet Metab Rep 2019 Jun 20;19:100460. Epub 2019 Feb 20.

Department of Pediatrics, Division of Medical Genetics & Genomic Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.

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http://dx.doi.org/10.1016/j.ymgmr.2019.100460DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6383325PMC
June 2019

Early progression of Krabbe disease in patients with symptom onset between 0 and 5 months.

Orphanet J Rare Dis 2019 02 18;14(1):46. Epub 2019 Feb 18.

Program for the Study of Neurodevelopment in Rare Disorders and Center for Rare Disease Therapy, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh, 4401 Penn Avenue, Pittsburgh, PA, 15144, USA.

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http://dx.doi.org/10.1186/s13023-019-1018-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378723PMC
February 2019

Consensus guidelines for newborn screening, diagnosis and treatment of infantile Krabbe disease.

Orphanet J Rare Dis 2018 02 1;13(1):30. Epub 2018 Feb 1.

Wadsworth Center, New York State Department of Health, Newborn Screening Program, David Axelrod Institute, 120 New Scotland Ave., Albany, NY, 12201, USA.

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http://dx.doi.org/10.1186/s13023-018-0766-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5796396PMC
February 2018

Krabbe disease: One Hundred years from the bedside to the bench to the bedside.

J Neurosci Res 2016 11;94(11):982-9

Department of Neurology, Sidney Kimmel College of Medicine at Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1002/jnr.23743DOI Listing
November 2016

Expression of individual mutations and haplotypes in the galactocerebrosidase gene identified by the newborn screening program in New York State and in confirmed cases of Krabbe's disease.

J Neurosci Res 2016 11;94(11):1076-83

Lysosomal Diseases Testing Laboratory, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1002/jnr.23905DOI Listing
November 2016

A Patient With Atypical Multiple Sulfatase Deficiency.

Pediatr Neurol 2016 Apr 24;57:98-100. Epub 2015 Dec 24.

Section of Neurology, St Christopher's Hospital for Children, Philadelphia, Pennsylvania; Department of Pediatrics, Drexel University College of Medicine, Philadelphia, Pennsylvania. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2015.10.023DOI Listing
April 2016

Long-term Improvements in Lifespan and Pathology in CNS and PNS After BMT Plus One Intravenous Injection of AAVrh10-GALC in Twitcher Mice.

Mol Ther 2015 Nov 2;23(11):1681-1690. Epub 2015 Sep 2.

Department of Neurology, Sidney Kimmel College of Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. Electronic address:

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http://dx.doi.org/10.1038/mt.2015.145DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4817953PMC
November 2015

Enzyme replacement therapy of a novel humanized mouse model of globoid cell leukodystrophy.

Exp Neurol 2015 Sep 6;271:36-45. Epub 2015 May 6.

Rheinische Friedrich-Wilhelms University, Institute of Biochemistry and Molecular Biology, Nussallee 11, D-53115 Bonn, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.expneurol.2015.04.020DOI Listing
September 2015

Preclinical characterization of DUOC-01, a cell therapy product derived from banked umbilical cord blood for use as an adjuvant to umbilical cord blood transplantation for treatment of inherited metabolic diseases.

Cytotherapy 2015 Jun 12;17(6):803-815. Epub 2015 Mar 12.

Robertson Clinical and Translational Cell Therapy Program, Duke Translational Medicine Institute, Duke University Medical Center, Durham, North Carolina, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jcyt.2015.02.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4843803PMC
June 2015

Intravenous injection of AAVrh10-GALC after the neonatal period in twitcher mice results in significant expression in the central and peripheral nervous systems and improvement of clinical features.

Mol Genet Metab 2015 Mar 11;114(3):459-66. Epub 2014 Dec 11.

Department of Neurology, Sidney Kimmel College of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.1016/j.ymgme.2014.12.300DOI Listing
March 2015

Iminosugar-based galactoside mimics as inhibitors of galactocerebrosidase: SAR studies and comparison with other lysosomal galactosidases.

ChemMedChem 2014 Dec 5;9(12):2647-52. Epub 2014 Nov 5.

Institut de Chimie Organique et Analytique (ICOA), UMR 7311, Université d'Orléans & CNRS, Rue de Chartres, BP 6759, 45067 Orléans cedex 2 (France).

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http://dx.doi.org/10.1002/cmdc.201402411DOI Listing
December 2014

Krabbe disease: are certain mutations disease-causing only when specific polymorphisms are present or when inherited in trans with specific second mutations?

Mol Genet Metab 2014 Mar 19;111(3):307-308. Epub 2013 Dec 19.

Department of Neurology, Jefferson Medical College, 1020 Locust St., Room346, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.1016/j.ymgme.2013.12.009DOI Listing
March 2014

Lysosomal storage diseases: heterogeneous group of disorders.

Bioimpacts 2013 2;3(4):145-7. Epub 2013 Dec 2.

Department of Neurology, Jefferson Medical College, Philadelphia, Pennsylvanian, USA.

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http://dx.doi.org/10.5681/bi.2013.029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3892733PMC
January 2014

Sixteen novel mutations in the arylsulfatase A gene causing metachromatic leukodystrophy.

Gene 2013 Nov 31;530(2):323-8. Epub 2013 Aug 31.

Lysosomal Diseases Testing Laboratory, Department of Neurology, Thomas Jefferson University, 1020 Locust Street, Room 346, Philadelphia, PA 19107, USA. Electronic address:

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http://dx.doi.org/10.1016/j.gene.2013.08.065DOI Listing
November 2013

Extended normal life after AAVrh10-mediated gene therapy in the mouse model of Krabbe disease.

Mol Ther 2012 Nov 31;20(11):2031-42. Epub 2012 Jul 31.

Department of Neurology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1038/mt.2012.153DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3498799PMC
November 2012

Metachromatic leukodystrophy: a case of triplets with the late infantile variant and a systematic review of the literature.

J Child Neurol 2010 May 28;25(5):572-80. Epub 2009 Dec 28.

Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas, USA.

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http://dx.doi.org/10.1177/0883073809341669DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4301611PMC
May 2010

Effects of treatments on inflammatory and apoptotic markers in the CNS of mice with globoid cell leukodystrophy.

Brain Res 2009 Dec 11;1300:146-58. Epub 2009 Sep 11.

Department of Neurology, Jefferson Medical College, 1020 Locust street, Room #394, Philadelphia, PA 19107, USA.

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http://linkinghub.elsevier.com/retrieve/pii/S000689930901891
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http://dx.doi.org/10.1016/j.brainres.2009.09.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2785448PMC
December 2009

Brain proton magnetic resonance spectroscopy and neuromuscular pathology in a patient with GM1 gangliosidosis.

J Child Neurol 2008 Jan;23(1):73-8

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA.

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http://dx.doi.org/10.1177/0883073807307088DOI Listing
January 2008

Biochemical and pathological evaluation of long-lived mice with globoid cell leukodystrophy after bone marrow transplantation.

Mol Genet Metab 2005 Sep-Oct;86(1-2):150-9

Department of Neurology, Jefferson Medical College, 1020 Locust Street, Room 394, Philadelphia, PA, USA.

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http://dx.doi.org/10.1016/j.ymgme.2005.06.023DOI Listing
January 2006

Galactocerebrosidase-deficient oligodendrocytes maintain stable central myelin by exogenous replacement of the missing enzyme in mice.

Proc Natl Acad Sci U S A 2005 Dec 13;102(51):18670-5. Epub 2005 Dec 13.

Department of Medical Sciences, School of Veterinary Medicine, University of Wisconsin, Madison, WI 53706, USA.

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http://dx.doi.org/10.1073/pnas.0506473102DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1317926PMC
December 2005

Insulin-like growth factor-1 provides protection against psychosine-induced apoptosis in cultured mouse oligodendrocyte progenitor cells using primarily the PI3K/Akt pathway.

Mol Cell Neurosci 2005 Nov 19;30(3):398-407. Epub 2005 Sep 19.

Department of Neurology, 1020 Locust Street, Room 394, Jefferson Medical College, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.1016/j.mcn.2005.08.004DOI Listing
November 2005

AAV2/5 vector expressing galactocerebrosidase ameliorates CNS disease in the murine model of globoid-cell leukodystrophy more efficiently than AAV2.

Mol Ther 2005 Sep;12(3):422-30

Department of Internal Medicine, Washington University School of Medicine, Box 8007, 660 South Euclid Avenue, St. Louis, MO 63110, USA.

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http://dx.doi.org/10.1016/j.ymthe.2005.04.019DOI Listing
September 2005

Distinguishing Pacman dysplasia from mucolipidosis II: comment on Saul et al. [2005].

Am J Med Genet A 2005 Jun;135(3):333

Medical Genetics Institute, Cedars-Sinai Medical Center, Los Angeles, California 90048, USA.

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http://dx.doi.org/10.1002/ajmg.a.30717DOI Listing
June 2005

Transplantation of umbilical-cord blood in babies with infantile Krabbe's disease.

N Engl J Med 2005 May;352(20):2069-81

Program for Neurodevelopmental Function in Rare Disorders, Clinical Center for the Study of Development and Learning, University of North Carolina at Chapel Hill, Chapel Hill 27599-7255, USA.

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http://dx.doi.org/10.1056/NEJMoa042604DOI Listing
May 2005

Cord-blood transplants from unrelated donors in patients with Hurler's syndrome.

N Engl J Med 2004 May;350(19):1960-9

Pediatric Stem Cell Transplant Program, Duke University Medical Center, Durham, NC 27710, USA.

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http://dx.doi.org/10.1056/NEJMoa032613DOI Listing
May 2004

Psychosine-induced apoptosis in a mouse oligodendrocyte progenitor cell line is mediated by caspase activation.

Neurosci Lett 2004 Apr;358(3):205-9

Department of Neurology, 1020 Locust Street, Room 394, Jefferson Medical College, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.1016/j.neulet.2003.12.126DOI Listing
April 2004

Cell-type-specific gene delivery into neuronal cells in vitro and in vivo.

Virology 2003 Sep;314(1):74-83

The Dorrance H. Hamilton Laboratories, Division of Infectious Diseases, Center for Human Virology and Biodefense, and Department of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.1016/s0042-6822(03)00402-1DOI Listing
September 2003

Disease-causing mutations in cis with the common arylsulfatase A pseudodeficiency allele compound the difficulties in accurately identifying patients and carriers of metachromatic leukodystrophy.

Mol Genet Metab 2003 Jun;79(2):83-90

Lysosomal Diseases Testing Laboratory, Department of Neurology, Jefferson Medical College, 1020 Locust St., Room 394, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.1016/s1096-7192(03)00076-3DOI Listing
June 2003

Mutation analysis of feline Niemann-Pick C1 disease.

Mol Genet Metab 2003 Jun;79(2):99-103

Colorado State University, Fort Collins, CO, USA.

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http://dx.doi.org/10.1016/s1096-7192(03)00074-xDOI Listing
June 2003

Lysosomal storage disorders: diagnostic dilemmas and prospects for therapy.

Genet Med 2002 Nov-Dec;4(6):412-9

Department of Neurology, Jefferson Medical College, Philadelphia, Pennsylvania 19107, USA.

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http://dx.doi.org/10.1097/00125817-200211000-00003DOI Listing
May 2003

Insights into the diagnosis and treatment of lysosomal storage diseases.

Arch Neurol 2003 Mar;60(3):322-8

Department of Neurology, Jefferson Medical College, 1020 Locust St, Room 394, Philadelphia, PA 19107, USA.

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http://dx.doi.org/10.1001/archneur.60.3.322DOI Listing
March 2003

An autoantibody to GAD65 in sera of patients with juvenile neuronal ceroid lipofuscinoses.

Neurology 2002 Dec;59(11):1816-7

Center for Aging and Developmental Biology, University of Rochester School of Medicine and Dentistry, NY, USA.

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http://dx.doi.org/10.1212/01.wnl.0000041913.97883.8bDOI Listing
December 2002

Niemann-pick disease type C in neonatal cholestasis at a North American Center.

J Pediatr Gastroenterol Nutr 2002 Jul;35(1):44-50

Pediatric Liver Center and Liver Transplantation Program, Department of Pediatrics, University of Colorado School of Medicine and the Children's Hospital, Denver, Colorado 80218, USA.

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http://dx.doi.org/10.1097/00005176-200207000-00011DOI Listing
July 2002