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51

Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: A case series

D. A. Shellmer, A. DeVito Dabbs, M. A. Dew, R. B. Noll, H. Feldman, K. A. Strauss, D. H. Morton, J. Vockley, G. V. Mazariegos

Journal:

Pediatric Transplantation

Year:

2011

Language:

english

File:

PDF, 202 KB

english, 2011

52

Genome-wide gene expression of the rare, malignant Reed-Sternberg cell of Hodgkin lymphoma

Cossman, Jeffrey, Vockley, Joseph, Carter, Kenneth, Ruben, Steven, Staudt, Louis, Barash, Steven, Birse, Charles, Rosen, Craig, Dolginow, Doug, Lennon, Greg

Journal:

Nature Genetics

Year:

1999

Language:

english

File:

PDF, 112 KB

english, 1999

53

Increased Levels of Plasma Acylcarnitines in Obesity and Type 2 Diabetes and Identification of a Marker of Glucolipotoxicity

Stephanie J. Mihalik, Bret H. Goodpaster, David E. Kelley, Donald H. Chace, Jerry Vockley, Frederico G.S. Toledo, James P. DeLany

Journal:

Year:

2010

Language:

english

File:

PDF, 165 KB

english, 2010

54

The human arginases and arginase deficiency

R. Iyer, C. P. Jenkinson, J. G. Vockley, R. M. Kern, W. W. Grody, S. Cederbaum

Journal:

Journal of Inherited Metabolic Disease

Year:

1998

Language:

english

File:

PDF, 284 KB

english, 1998

55

p73 mutations are not detected in sporadic and hereditary breast cancer

David I. Schwartz, Noralane M. Lindor, Cate Walsh-Vockley, Patrick C. Roche, Ming Mai, David I. Smith, Wanguo Liu, Fergus J. Couch

Journal:

Breast Cancer Research and Treatment

Year:

1999

Language:

english

File:

PDF, 39 KB

english, 1999

56

Loss of Function Mutations in Conserved Regions of the Human Arginase I Gene

Joseph G. Vockley, Barbara K. Goodman, David E. Tabor, Rita M. Kern, Christopher P. Jenkinson, Wayne W. Grody, Stephen D. Cederbaum

Journal:

Biochemical and Molecular Medicine

Year:

1996

Language:

english

File:

PDF, 477 KB

english, 1996

57

Functional and molecular analysis of liver arginase promoter sequences from man andMacaca fascicularis

Barbara K. Goodman, Deborah Klein, David E. Tabor, Joseph G. Vockley, Stephen D. Cederbaum, Wayne W. Grody

Journal:

Somatic Cell and Molecular Genetics

Year:

1994

Language:

english

File:

PDF, 1.44 MB

english, 1994

58

Delivery of cytosolic liver arginase into the mitochondrial matrix space: A possible novel site for gene replacement therapy

Paul B. Wissmann, Barbara K. Goodman, Joseph G. Vockley, Rita M. Kern, Stephen D. Cederbaum, Wayne W. Grody

Journal:

Somatic Cell and Molecular Genetics

Year:

1996

Language:

english

File:

PDF, 1.11 MB

english, 1996

59

Normal N-acetylglutamate concentration measured in liver from a new patient with N-acetylglutamate synthetase deficiency: Physiologic and biochemical implications

Jerry Vockley, Catherine M.Walsh Vockley, Shaun-Pei Lin, Mendel Tuchman, Tzee-Chung Wu, Ching-Yuang Lin, Margretta R. Seashore

Journal:

Biochemical Medicine and Metabolic Biology

Year:

1992

Language:

english

File:

PDF, 6.13 MB

english, 1992

60

Liver Transplantation for Classical Maple Syrup Urine Disease: Long-Term Follow-Up in 37 Patients and Comparative United Network for Organ Sharing Experience

George V. Mazariegos, D. Holmes Morton, Rakesh Sindhi, Kyle Soltys, Navdeep Nayyar, Geoffrey Bond, Diana Shellmer, Benjamin Shneider, Jerry Vockley, Kevin A. Strauss

Journal:

The Journal of Pediatrics

Year:

2012

Language:

english

File:

PDF, 490 KB

english, 2012

61

Identification of the catalytic residue of human short/branched chain acyl-CoA dehydrogenase by in vitro mutagenesis

Barbara Binzak, Jan Willard, Jerry Vockley

Journal:

Biochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology

Year:

1998

Language:

english

File:

PDF, 204 KB

english, 1998

62

Elective Liver Transplantation for the Treatment of Classical Maple Syrup Urine Disease

K. A. Strauss, G. V. Mazariegos, R. Sindhi, R. Squires, D. N. Finegold, G. Vockley, D. L. Robinson, C. Hendrickson, M. Virji, L. Cropcho, E. G. Puffenberger, W. McGhee, L. M. Seward, D. H. Morton

Journal:

American Journal of Transplantation

Year:

2006

Language:

english

File:

PDF, 313 KB

english, 2006

63

Cloning of a cDNA for Short/Branched Chain Acyl-Coenzyme A Dehydrogenase from Rat and Characterization of Its Tissue Expression and Substrate Specificity

Jan Willard, Caroline Vicanek, Kevin P. Battaile, Paul P. Van Veldhoven, Abdul H. Fauq, Rima Rozen, Jerry Vockley

Journal:

Archives of Biochemistry and Biophysics

Year:

1996

Language:

english

File:

PDF, 280 KB

english, 1996

64

Microelectrospray Ionization Analysis of Noncovalent Interactions within the Electron Transferring Flavoprotein

Heidi M. Hoard, Linda M. Benson, Jerry Vockley, Stephen Naylor

Journal:

Biochemical and Biophysical Research Communications

Year:

2001

Language:

english

File:

PDF, 126 KB

english, 2001

65

Isolation and Expression of a cDNA Encoding the Precursor for a Novel Member (ACADSB) of the Acyl-CoA Dehydrogenase Gene Family

Rima Rozen, Jerry Vockley, Lubing Zhou, Renate Milos, Jan Willard, Katherine Fu, Caroline Vicanek, Lawrence Low-Nang, Elena Torban, Beatrice Fournier

Journal:

Year:

1994

File:

PDF, 553 KB

1994

66

Cloning and Characterization of the Human Type II Arginase Gene

Joseph G. Vockley, Christopher P. Jenkinson, Hridayabiranjan Shukla, Rita M. Kern, Wayne W. Grody, Stephen D. Cederbaum

Journal:

Year:

1996

Language:

english

File:

PDF, 1.36 MB

english, 1996

67

Molecular Basis of Hyperargininemia: Structure-Function Consequences of Mutations in Human Liver Arginase

David E. Ash, Laura R. Scolnick, Zoltan F. Kanyo, Joseph G. Vockley, Stephen D. Cederbaum, David W. Christianson

Journal:

Molecular Genetics and Metabolism

Year:

1998

Language:

english

File:

PDF, 459 KB

english, 1998

68

Cloning and Characterization of the Mouse and Rat Type II Arginase Genes

Ramaswamy K. Iyer, Joanne M. Bando, Christopher P. Jenkinson, Joseph G. Vockley, Phillip S. Kim, Rita M. Kern, Stephen D. Cederbaum, Wayne W. Grody

Journal:

Molecular Genetics and Metabolism

Year:

1998

Language:

english

File:

PDF, 853 KB

english, 1998

69

Structure and Analysis of the Human Dimethylglycine Dehydrogenase Gene

Barbara A. Binzak, Joseph G. Vockley, Robert B. Jenkins, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2000

Language:

english

File:

PDF, 85 KB

english, 2000

70

Identification of Caenorhabditis elegans Isovaleryl-CoA Dehydrogenase and Structural Comparison with Other Acyl-CoA Dehydrogenases

Al-Walid A. Mohsen, Bryan Navarette, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2001

Language:

english

File:

PDF, 440 KB

english, 2001

71

Arginine 387 of Human Isovaleryl-CoA Dehydrogenase Plays a Crucial Role in Substrate/Product Binding

Samuel L. Volchenboum, Al-Walid A. Mohsen, Jung-Ja P. Kim, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2001

Language:

english

File:

PDF, 284 KB

english, 2001

72

Epitopes of human intestinal alkaline phosphatases, defined by monoclonal antibodies

Kunal Ray, Jerry Vockley, Harry Harris

Journal:

Year:

1984

Language:

english

File:

PDF, 437 KB

english, 1984

73

High-level expression of an altered cDNA encoding human isovaleryl-CoA dehydrogenase in Escherichia coli

Al-Walid A. Mohsen, Jerry Vockley

Journal:

Year:

1995

Language:

english

File:

PDF, 471 KB

english, 1995

74

Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase: study of the effect of genetic defects on enzyme stability

Ibrahim Nasser, Al-Walid Mohsen, Ilian Jelesarov, Jerry Vockley, Peter Macheroux, Sandro Ghisla

Journal:

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

Year:

2004

Language:

english

File:

PDF, 941 KB

english, 2004

75

Brown adipose tissue function in short-chain acyl-CoA dehydrogenase deficient mice

Helen Skilling, Paul M. Coen, Liane Fairfull, Robert E. Ferrell, Bret H. Goodpaster, Jerry Vockley, Eric S. Goetzman

Journal:

Biochemical and Biophysical Research Communications

Year:

2010

Language:

english

File:

PDF, 330 KB

english, 2010

76

Barriers to the successful treatment of liver disease by hepatocyte transplantation

Kyle A. Soltys, Alejandro Soto-Gutiérrez, Masaki Nagaya, Kevin M. Baskin, Melvin Deutsch, Ryotaro Ito, Benjamin L. Shneider, Robert Squires, Jerry Vockley, Chandan Guha, Jayanta Roy-Chowdhury, Stephen

Journal:

Journal of Hepatology

Year:

2010

Language:

english

File:

PDF, 915 KB

english, 2010

77

Novel mutation in MYH7 gene associated with distal myopathy and cardiomyopathy

Houman Homayoun, Simin Khavandgar, Jacqueline M. Hoover, Al-Walid Mohsen, Jerry Vockley, David Lacomis, Paula R. Clemens

Journal:

Neuromuscular Disorders

Year:

2011

Language:

english

File:

PDF, 349 KB

english, 2011

78

Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid β-oxidation

A. Michele Schuler, Barbara A. Gower, Dietrich Matern, Piero Rinaldo, Jerry Vockley, Philip A. Wood

Journal:

Molecular Genetics and Metabolism

Year:

2005

Language:

english

File:

PDF, 369 KB

english, 2005

79

In vitro correction of medium chain acyl CoA dehydrogenase deficiency with a recombinant adenoviral vector

David B. Schowalter, Dietrich Matern, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2005

Language:

english

File:

PDF, 817 KB

english, 2005

80

In Memoriam

Piero Rinaldo, Paul M. Coates, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2005

Language:

english

File:

PDF, 111 KB

english, 2005

81

Functional analysis of acyl-CoA dehydrogenase catalytic residue mutants using surface plasmon resonance and circular dichroism

Eric S. Goetzman, Miao He, Tien V. Nguyen, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2006

Language:

english

File:

PDF, 473 KB

english, 2006

82

In vitro characterization and in vivo expression of human very-long chain acyl-CoA dehydrogenase

J. Lawrence Merritt II, Dietrich Matern, Jerry Vockley, Jan Daniels, Tien V. Nguyen, David B. Schowalter

Journal:

Molecular Genetics and Metabolism

Year:

2006

Language:

english

File:

PDF, 416 KB

english, 2006

83

Different spectrum of mutations of isovaleryl-CoA dehydrogenase (IVD) gene in Korean patients with isovaleric acidemia

Yong-Wha Lee, Dong Hwan Lee, Jerry Vockley, Nam-Doo Kim, You Kyoung Lee, Chang-Seok Ki

Journal:

Molecular Genetics and Metabolism

Year:

2007

Language:

english

File:

PDF, 1.62 MB

english, 2007

84

Expression and characterization of mutations in human very long-chain acyl-CoA dehydrogenase using a prokaryotic system

Eric S. Goetzman, Yudong Wang, Miao He, Al-Walid Mohsen, Brittani K. Ninness, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2007

Language:

english

File:

PDF, 1.15 MB

english, 2007

85

Newborn screening: After the thrill is gone

Journal:

Molecular Genetics and Metabolism

Year:

2007

Language:

english

File:

PDF, 148 KB

english, 2007

86

Glutaric aciduria type 2 and newborn screening: Commentary

Journal:

Molecular Genetics and Metabolism

Year:

2008

Language:

english

File:

PDF, 87 KB

english, 2008

87

Short-chain acyl-coenzyme A dehydrogenase deficiency

Reena Jethva, Michael J. Bennett, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2008

Language:

english

File:

PDF, 168 KB

english, 2008

88

Clinical trials: Curing a critical deficiency in metabolic medicine

Jerry Vockley, Catherine M. Walsh Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2010

Language:

english

File:

PDF, 115 KB

english, 2010

89

Characterization of new ACADSB gene sequence mutations and clinical implications in patients with 2-methylbutyrylglycinuria identified by newborn screening

Jaffar Alfardan, Al-Walid Mohsen, Sara Copeland, Jay Ellison, Laura Keppen-Davis, Marianne Rohrbach, Berkley R. Powell, Jane Gillis, Dietrich Matern, Jeffrey Kant, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2010

Language:

english

File:

PDF, 573 KB

english, 2010

90

Low expression of long-chain acyl-CoA dehydrogenase in human skeletal muscle

Amy C. Maher, Al-Walid Mohsen, Jerry Vockley, Mark A. Tarnopolsky

Journal:

Molecular Genetics and Metabolism

Year:

2010

Language:

english

File:

PDF, 248 KB

english, 2010

91

Identification and characterization of new long chain Acyl-CoA dehydrogenases

Miao He, Zhengtong Pei, Al-Walid Mohsen, Paul Watkins, Geoffrey Murdoch, Paul P. Van Veldhoven, Regina Ensenauer, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2011

Language:

english

File:

PDF, 2.08 MB

english, 2011

92

Thoroughly modern medicine

Georgianne L. Arnold, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2011

Language:

english

File:

PDF, 187 KB

english, 2011

93

Human long chain, very long chain and medium chain acyl-CoA dehydrogenases are specific for the S-enantiomer of 2- methylpentadecanoyl-CoA

Kevin P Battaile, Martha McBurney, Paul P Van Veldhoven, Jerry Vockley

Journal:

Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism

Year:

1998

Language:

english

File:

PDF, 65 KB

english, 1998

94

Severe hypoglycemia as a presenting symptom of carbohydrate-deficient glycoprotein syndrome

Dusica Babovic-Vuksanovic, Marc C. Patterson, W.Frederick Schwenk, John F. O’Brien, Jerry Vockley, Hudson H. Freeze, Darshini P. Mehta, Virginia V. Michels

Journal:

The Journal of Pediatrics

Year:

1999

Language:

english

File:

PDF, 69 KB

english, 1999

95

Cloning of genomic and cDNA for mouse isovaleryl-CoA dehydrogenase (IVD) and evolutionary comparison to other known IVDs

Jan Marie Willard, Thomas Reinard, Al-Walid Mohsen, Jerry Vockley

Journal:

Year:

2001

Language:

english

File:

PDF, 310 KB

english, 2001

96

Defects of mitochondrial β-oxidation: a growing group of disorders

Jerry Vockley, David A.H. Whiteman

Journal:

Neuromuscular Disorders

Year:

2002

Language:

english

File:

PDF, 245 KB

english, 2002

97

A novel mitochondrial tRNALeu(UUR) mutation in a patient with features of MERRF and Kearns–Sayre syndrome

Yutaka Nishigaki, Saba Tadesse, Eduardo Bonilla, Dikoma Shungu, Stephen Hersh, Bronya J.B Keats, Charles I Berlin, Morton F Goldberg, Jerry Vockley, Salvatore DiMauro, Michio Hirano

Journal:

Neuromuscular Disorders

Year:

2003

Language:

english

File:

PDF, 409 KB

english, 2003

98

Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans

Tien V Nguyen, Brage S Andresen, Thomas J Corydon, Sandro Ghisla, Nasser Abd-El Razik, Al-Walid A Mohsen, Stephen D Cederbaum, Diane S Roe, Charles R Roe, Nicolas J Lench, Jerry Vockley

Journal:

Molecular Genetics and Metabolism

Year:

2002

Language:

english

File:

PDF, 644 KB

english, 2002

99

Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial β-oxidation of unsaturated fatty acids

Weiping Le, Azfar S. Abbas, Howard Sprecher, Jerry Vockley, Horst Schulz

Journal:

Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids

Year:

2000

Language:

english

File:

PDF, 240 KB

english, 2000

100

Short-chain hydroxyacyl–coenzyme A dehydrogenase deficiency presenting as unexpected infant death: A family study

Eileen P. Treacy, Deborah M. Lambert, Robert Barnes, Richard L. Boriack, Jerry Vockley, Laurie K. O'Brien, Patricia M. Jones, Michael J. Bennett

Journal:

The Journal of Pediatrics

Year:

2000

Language:

english

File:

PDF, 48 KB

english, 2000

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