1963 …2019
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Research Output 1963 2019

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Article
2019
Open Access
Hydroxyurea
Down Syndrome
Episodic Memory
Nootropic Agents
Unfolded Protein Response
2018

Hydroxyurea attenuates oxidative, metabolic, and excitotoxic stress in rat hippocampal neurons and improves spatial memory in a mouse model of Alzheimer's disease

Brose, R. D., Lehrmann, E., Zhang, Y., Reeves, R. H., Smith, K. D. & Mattson, M. P., Dec 1 2018, In : Neurobiology of Aging. 72, p. 121-133 13 p.

Research output: Contribution to journalArticle

Hydroxyurea
Alzheimer Disease
Neurons
Hydrogen Peroxide
Cell Respiration
2014

Sulforaphane treatment of autism spectrum disorder (ASD)

Singh, K., Connors, S. L., Macklin, E. A., Smith, K. D., Fahey, J. W., Talalay, P. & Zimmerman, A. W., Oct 28 2014, In : Proceedings of the National Academy of Sciences of the United States of America. 111, 43, p. 15550-15555 6 p.

Research output: Contribution to journalArticle

Placebos
Checklist
Brassica
Phytochemicals
Therapeutics
2012

Activation of the stress proteome as a mechanism for small molecule therapeutics

Brose, R. D., Shin, G., Mcguinness, M. C., Schneidereith, T., Purvis, S., Dong, G. X., Keefer, J., Spencer, F. & Smith, K. D., Oct 2012, In : Human Molecular Genetics. 21, 19, p. 4237-4252 16 p., dds247.

Research output: Contribution to journalArticle

Organelle Biogenesis
Proteome
Histone Deacetylase Inhibitors
Hydroxyurea
Therapeutic Uses

SOD2 as a potential modifier of X-linked adrenoleukodystrophy clinical phenotypes

Brose, R. D., Avramopoulos, D. & Smith, K. D., Jul 2012, In : Journal of Neurology. 259, 7, p. 1440-1447 8 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Phenotype
Demyelinating Diseases
Haplotypes
Valine
2009

The role of animal models in evaluating reasonable safety and efficacy for human trials of cell-based interventions for neurologic conditions

Regenberg, A., Mathews, D. J., Blass, D. M., Bok, H., Coyle, J. T., Duggan, P., Faden, R. R., Finkel, J., Gearhart, J. D., Hillis-Trupe, A., Hoke, A., Johnson, R., Johnston, M. V., Kahn, J., Kerr, D., King, P., Kurtzberg, J., Liao, S. M., McDonald, J. W., McKhann, G. & 11 others, Nelson, K. B., Rao, M., Siegel, A. W., Smith, K. D., Solter, D., Song, H., Sugarman, J., Vescovi, A., Young, W., Greely, H. T. & Traystman, R. J., Jan 2009, In : Journal of Cerebral Blood Flow and Metabolism. 29, 1, p. 1-9 9 p.

Research output: Contribution to journalArticle

Nervous System
Animal Models
Safety
Regenerative Medicine
Lenses

Unintended changes in cognition, mood, and behavior arising from cell-based interventions for neurological conditions: Ethical challenges

Duggan, P. S., Siegel, A. W., Blass, D. M., Bok, H., Coyle, J. T., Faden, R. R., Finkel, J., Gearhart, J. D., Greely, H. T., Hillis-Trupe, A., Hoke, A., Johnson, R., Johnston, M. V., Kahn, J., Kerr, D., King, P., Kurtzberg, J., Liao, S. M., McDonald, J. W., McKhann, G. & 12 others, Nelson, K. B., Rao, M., Regenberg, A., Smith, K. D., Solter, D., Song, H., Sugarman, J., Traystman, R. J., Vescovi, A., Yanofski, J., Young, W. & Mathews, D. J., May 2009, In : American Journal of Bioethics. 9, 5, p. 31-36 6 p.

Research output: Contribution to journalArticle

Cognition
Ethical Analysis
Informed Consent
Self Concept
Central Nervous System
2008

Cell-based interventions for neurologic conditions: Ethical challenges for early human trials

Mathews, D. J., Sugarman, J., Bok, H., Blass, D. M., Coyle, J. T., Duggan, P., Finkel, J., Greely, H. T., Hillis-Trupe, A., Hoke, A., Johnson, R., Johnston, M. V., Kahn, J., Kerr, D., Kurtzberg, J., Liao, S. M., McDonald, J. W., McKhann, G., Nelson, K. B., Rao, M. & 9 others, Regenberg, A., Siegel, A. W., Smith, K. D., Solter, D., Song, H., Vescovi, A., Young, W., Gearhart, J. D. & Faden, R. R., Jul 22 2008, In : Neurology. 71, 4, p. 288-293 6 p.

Research output: Contribution to journalArticle

Ethics
Nervous System
Stem Cell Research
Institutional Practice
Research
2007

The role of peroxisomal ABC transporters in the mouse adrenal gland: The loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage

Lu, J. F., Barron-Casella, E., Deering, R., Heinzer, A. K., Moser, A. B., DeMesy Bentley, K. L., Wand, G. S., McGuinness, M. C., Pei, Z., Watkins, P. A., Pujol, A., Smith, K. D. & Powers, J. M., Mar 2007, In : Laboratory Investigation. 87, 3, p. 261-272 12 p.

Research output: Contribution to journalArticle

ATP-Binding Cassette Transporters
Adrenal Glands
Knockout Mice
Oxidative Stress
Hemizygote
2006

Role of Cyclic Nucleotides in Fetal Hemoglobin Induction in Cultured CD34+ Cells

Keefer, J., Schneidereith, T. A., Mays, A., Purvis, S. H., Dover, G. J. & Smith, K. D., Sep 2006, In : Experimental Hematology. 34, 9, p. 1150-1160 11 p.

Research output: Contribution to journalArticle

Azacitidine
Fetal Hemoglobin
Butyric Acid
Hydroxyurea
Cyclic Nucleotides

Role of cyclic nucleotides in fetal hemoglobin induction in cultured CD34+ cells.

Keefer, J. R., Schneidereith, T. A., Mays, A., Purvis, S. H., Dover, G. J. & Smith, K. D., Sep 2006, In : Experimental Hematology. 34, 9, p. 1151-1161 11 p.

Research output: Contribution to journalArticle

Azacitidine
Fetal Hemoglobin
Butyric Acid
Hydroxyurea
Cyclic Nucleotides
2005

Adreno-leukodystrophy: Oxidative stress of mice and men

Powers, J. M., Pei, Z., Heinzer, A. K., Deering, R., Moser, A. B., Moser, H. W., Watkins, P. A. & Smith, K. D., Dec 2005, In : Journal of Neuropathology and Experimental Neurology. 64, 12, p. 1067-1079 13 p.

Research output: Contribution to journalArticle

Adrenal Cortex
Oxidative Stress
Knockout Mice
Demyelinating Diseases
Phenotype
2004

X-linked adrenoleukodystrophy: Role of very long-chain acyl-CoA synthetases

Jia, Z., Pei, Z., Li, Y., Wei, L., Smith, K. D. & Watkins, P. A., Sep 2004, In : Molecular Genetics and Metabolism. 83, 1-2, p. 117-127 11 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Fatty Acids
Cholesterol Esters
Pathology
Enzymes
2003

A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-linked adrenoleukodystrophy

Heinzer, A. K., Watkins, P. A., Lu, J. F., Kemp, S., Moser, A. B., Li, Y. Y., Mihalik, S., Powers, J. M. & Smith, K. D., May 15 2003, In : Human Molecular Genetics. 12, 10, p. 1145-1154 10 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Knockout Mice
Fatty Acids
Peroxisomes
long-chain-fatty-acid-CoA ligase

Mouse models and genetic modifiers in X-linked adrenoleukodystrophy

Heinzer, A. K., Mcguinness, M. C., Lu, J. F., Stine, O. C., Wei, H., Van Der Vlies, M., Dong, G., Powers, J., Watkins, P. A. & Smith, K. D., 2003, In : Advances in Experimental Medicine and Biology. 544, p. 75-93 19 p.

Research output: Contribution to journalArticle

Mitochondria
ATP-Binding Cassette Transporters
Animals
Fatty Acids

Role of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophy

McGuinness, M. C., Lu, J. F., Zhang, H. P., Dong, G. X., Heinzer, A. K., Watkins, P. A., Powers, J. & Smith, K. D., Jan 2003, In : Molecular and Cellular Biology. 23, 2, p. 744-753 10 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Mitochondria
Fatty Acids
Fibroblasts
Peroxisomal Disorders

The Acyl-CoA synthetase "bubblegum" (lipidosin): Further characterization and role in neuronal fatty acid β-oxidation

Pei, Z., Oey, N. A., Zuidervaart, M. M., Jia, Z., Li, Y., Steinberg, S. J., Smith, K. D. & Watkins, P. A., Nov 21 2003, In : Journal of Biological Chemistry. 278, 47, p. 47070-47078 9 p.

Research output: Contribution to journalArticle

Coenzyme A Ligases
Fatty Acids
Oxidation
Mitochondria
Metabolism
2002

Contiguous deletion of the X-linked adrenoleukodystrophy gene (ABCD1) and DXS1357E: A novel neonatal phenotype similar to peroxisomal biogenesis disorders

Corzo, D., Gibson, W., Johnson, K., Mitchell, G., LePage, G., Cox, G. F., Casey, R., Zeiss, C., Tyson, H., Cutting, G. R., Raymond, G. V., Smith, K. D., Watkins, P. A., Moser, A. B., Moser, H. W. & Steinberg, S. J., 2002, In : American Journal of Human Genetics. 70, 6, p. 1520-1531 12 p.

Research output: Contribution to journalArticle

Peroxisomal Disorders
Adrenoleukodystrophy
X-Linked Genes
Phenotype
Mutation

Mouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophy.

Heinzer, A. K., Kemp, S., Lu, J. F., Watkins, P. A. & Smith, K. D., Aug 9 2002, In : Journal of Biological Chemistry. 277, 32, p. 28765-28773 9 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Fatty Acids
Peroxisomes
Genes
Enzymes

Participation of two members of the very long-chain acyl-CoA synthetase family in bile acid synthesis and recycling

Mihalik, S. J., Steinberg, S. J., Pei, Z., Park, J., Kim, D. G., Heinzer, A. K., Dacremont, G., Wanders, R. J. A., Cuebas, D. A., Smith, K. D. & Watkins, P. A., Jul 5 2002, In : Journal of Biological Chemistry. 277, 27, p. 24771-24779 9 p.

Research output: Contribution to journalArticle

Recycling
Bile Acids and Salts
Cholates
Coenzyme A
Metabolism

PEX11α is required for peroxisome proliferation in response to 4-phenylbutyrate but is dispensable for peroxisome proliferator-activated receptor alpha-mediated peroxisome proliferation

Li, X., Baumgart, E., Dong, G. X., Morrell, J. C., Jimenez-Sanchez, G., Valle, D., Smith, K. D. & Gould, S. J., Dec 2002, In : Molecular and Cellular Biology. 22, 23, p. 8226-8240 15 p.

Research output: Contribution to journalArticle

PPAR alpha
Peroxisomes
4-phenylbutyric acid
Genes
Eukaryota
2001

Adrenoleukodystrophy: Incidence, new mutation rate, and results of extended family screening

Bezman, L., Moser, A. B., Raymond, G. V., Rinaldo, P., Watkins, P. A., Smith, K. D., Kass, N. E. & Moser, H. W., 2001, In : Annals of Neurology. 49, 4, p. 512-517 6 p.

Research output: Contribution to journalArticle

Hemizygote
Adrenoleukodystrophy
Mutation Rate
Incidence
Heterozygote

Evaluation of pharmacological induction of fatty acid β-oxidation in X-linked adrenoleukodystrophy

McGuinness, M. C., Zhang, H. P. & Smith, K. D., 2001, In : Molecular Genetics and Metabolism. 74, 1-2, p. 256-263 8 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Fatty Acids
Pharmacology
Oxidation
Fibroblasts
Oligodendroglia
Antigen Presentation
Adrenoleukodystrophy
Demyelinating Diseases
T-Lymphocytes

The role of heterocellular hereditary persistence of fetal haemoglobin in β0-thalassaemia intermedia

Chang, Y. P. C., Littera, R., Garau, R., Smith, K. D., Dover, G. J., Iannelli, S., Cacace, E. & Contu, L., 2001, In : British Journal of Haematology. 114, 4, p. 899-906 8 p.

Research output: Contribution to journalArticle

Fetal Hemoglobin
Globins
beta-Thalassemia
Thalassemia
Genotype
2000

Determination of 30 X-linked adrenoleukodystrophy mutations, including 15 not previously described

Lachtermacher, M. B. R., Seuánez, H. N., Moser, A. B., Moser, H. W. & Smith, K. D., 2000, In : Human Mutation. 15, 4, p. 348-353 6 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Mutation
Single-Stranded Conformational Polymorphism
Peroxisomes
Adrenal Cortex

Disruption of a Yeast Very-Long-Chain Acyl-CoA Synthetase Gene Simulates the Cellular Phenotype of X-Linked Adrenoleukodystrophy

Watkins, P. A., Lu, J. F., Braiterman, L. T., Steinberg, S. J. & Smith, K. D., 2000, In : Cell Biochemistry and Biophysics. 32, p. 333-337 5 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Yeast
Genes
Yeasts
Phenotype

One-step multiplex PCR strategy for identification of mutations by SSCP and DNA sequencing

Lachtermacher, M. B. R., Seuanez, H. N., Moser, H. W. & Smith, K. D., 2000, In : BioTechniques. 29, 2, p. 234-236 3 p.

Research output: Contribution to journalArticle

DNA Mutational Analysis
Adrenoleukodystrophy
Single-Stranded Conformational Polymorphism
Silver Staining
ATP-Binding Cassette Transporters

Pharmacological induction of peroxisomes in peroxisome biogenesis disorders

Wei, H., Kemp, S., McGuinness, M. C., Moser, A. B. & Smith, K. D., 2000, In : Annals of Neurology. 47, 3, p. 286-296 11 p.

Research output: Contribution to journalArticle

Peroxisomes
Infantile Refsum's Disease
Peroxisomal Disorders
Pharmacology
Zellweger Syndrome

Therapeutic developments in peroxisome biogenesis disorders

McGuinness, M. C., Wei, H. & Smith, K. D., 2000, In : Expert Opinion on Investigational Drugs. 9, 9, p. 1985-1992 8 p.

Research output: Contribution to journalArticle

Peroxisomes
Zellweger Syndrome
Plasmalogens
Diet Therapy
Fatty Acids

Very long-chain acyl-CoA synthetases: Human 'bubblegum' represents a new family of proteins capable of activating very long-chain fatty acids

Steinberg, S. J., Morgenthaler, J., Heinzer, A. K., Smith, K. D. & Watkins, P. A., Nov 10 2000, In : Journal of Biological Chemistry. 275, 45, p. 35162-35169 8 p.

Research output: Contribution to journalArticle

Coenzyme A Ligases
Fatty Acids
Proteins
COS Cells
Brain
1999

An Asian-Native American paternal lineage identified by RPS4Y resequencing and by microsatellite haplotyping

Bergen, A. W., Wang, C. Y., Tsai, J., Jefferson, K., Dey, C., Smith, K. D., Park, S. C., Tsai, S. J. & Goldman, D., Jan 1999, In : Annals of Human Genetics. 63, 1, p. 63-80 18 p.

Research output: Contribution to journalArticle

Asian Americans
North American Indians
Microsatellite Repeats
Y Chromosome
Population

Cerebral inflammation in X-linked adrenoleukodystrophy

McGuinness, M. C. & Smith, K. D., 1999, In : Archivum Immunologiae et Therapiae Experimentalis. 47, 5, p. 281-287 7 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Inflammation
Fatty Acids
Cholesterol Esters
Gangliosides

Mutational analysis and the pathogenesis of variant X-linked adrenoleukodystrophy phenotypes

Moser, H. W., Kemp, S. & Smith, K. D., 1999, In : Archives of Neurology. 56, 3, p. 273-275 3 p.

Research output: Contribution to journalArticle

Phenotype
Adrenoleukodystrophy
Fatty Acids
Oxidation
Peroxisomal Disorders
Acyl-CoA Oxidase

X-linked adrenoleukodystrophy: Genes, mutations, and phenotypes

Smith, K. D., Kemp, S., Braiterman, L. T., Lu, J. F., Wei, H. M., Geraghty, M., Stetten, G., Bergin, J. S., Pevsner, J. A. & Watkins, P. A., 1999, In : Neurochemical Research. 24, 4, p. 521-535 15 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
X-Linked Genes
Genes
Carrier Proteins
Phenotype
1998
Coenzyme A Ligases
Acyl Coenzyme A
Yeast
Saccharomyces cerevisiae
Fatty Acids

Gene redundancy and pharmacological gene therapy: Implications for X- linked adrenoleukodystrophy

Kemp, S., Wei, H. M., Lu, J. F., Braiterman, L. T., Mcguinness, M. C., Moser, A. B., Watkins, P. A. & Smith, K. D., Nov 1998, In : Nature Medicine. 4, 11, p. 1261-1268 8 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Gene therapy
Genetic Therapy
Redundancy
Fatty Acids

Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins

Braiterman, L. T., Zheng, S., Watkins, P. A., Geraghty, M. T., Johnson, G., McGuinness, M. C., Moser, A. B. & Smith, K. D., Feb 1998, In : Human Molecular Genetics. 7, 2, p. 239-247 9 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Carrier Proteins
Membrane Proteins
Fatty Acids
Adenosine Triphosphate
1997

A mouse model for X-linked adrenoleukodystrophy

Lu, J. F., Lawler, A. M., Watkins, P. A., Powers, J. M., Moser, A. B., Moser, H. W. & Smith, K. D., Aug 19 1997, In : Proceedings of the National Academy of Sciences of the United States of America. 94, 17, p. 9366-9371 6 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Fatty Acids
X-Linked Genes
Peroxisomal Disorders
Lipids

Human leukocyte antigens and cytokine expression in cerebral inflammatory demyelinative lesions of X-linked adrenoleukodystrophy and multiple sclerosis

McGuinness, M. C., Powers, J. M., Bias, W. B., Schmeckpeper, B. J., Segal, A. H., Gowda, V. C., Wesselingh, S. L., Berger, J., Griffin, D. & Smith, K. D., May 1997, In : Journal of Neuroimmunology. 75, 1-2, p. 174-182 9 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
HLA Antigens
Multiple Sclerosis
Cytokines
Histocompatibility Antigens Class II

The relative importance of the X-linked FCP locus and β-globin haplotypes in determining haemoglobin F levels: A study of SS patients homozygous for β(S) haplotypes

Chang, Y. P. C., Maier-Redelsperger, M., Smith, K. D., Contu, L., Ducrocq, R., De Montalembert, M., Belloy, M., Elion, J., Dover, G. J. & Girot, R., 1997, In : British Journal of Haematology. 96, 4, p. 806-814 9 p.

Research output: Contribution to journalArticle

Fetal Hemoglobin
Globins
Haplotypes
Central African Republic
Jamaica
1996

Expression of ALDP is altered in X-linked adrenoleukodystrophy

Watkins, P. A., Gould, S. J., Smith, M. A., Braiterman, L. T., Wei, H. M., Kok, F., Moser, B., Moser, H. W. & Smith, K. D., 1996, In : Annals of the New York Academy of Sciences. 804, p. 760-762 3 p.

Research output: Contribution to journalArticle

Functional studies of the X-linked adrenoleukodystrophy protein

Braiterman, L. T., Zheng, S., Watkins, P. A., Johnson, G., Moser, A. B., Moser, H. W. & Smith, K. D., 1996, In : Annals of the New York Academy of Sciences. 804, p. 763-764 2 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Proteins
Protein
1995

Adrenoleukodystrophy: Molecular genetics, pathology, and Lorenzo's oil

Moser, H. W., Powers, J. M. & Smith, K. D., 1995, In : Brain Pathology. 5, 3, p. 259-266 8 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Molecular Pathology
Molecular Biology
Clinical Pathology
Motion Pictures

Altered expression of ALDP in X-linked adrenoleukodystrophy

Watkins, P. A., Gould, S. J., Smith, M. A., Braiterman, L. T., Wei, H. M., Kok, F., Moser, A. B., Moser, H. W. & Smith, K. D., 1995, In : American Journal of Human Genetics. 57, 2, p. 292-301 10 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Missense Mutation
Mutation
Fatty Acids
Fibroblasts
Fetal Hemoglobin
Globins
Sickle Cell Anemia
Haplotypes
Genes

Mutational analysis of patients with X-linked adrenoleukodystrophy

Kok, F., Neumann, S., Sarde, C. O., Zheng, S., Wu, K. H., Wei, H. M., Bergin, J., Watkins, P. A., Gould, S. J., Sack, G. H., Moser, H., Mandel, J. L. & Smith, K. D., 1995, In : Human Mutation. 6, 2, p. 104-115 12 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Mutation
Genes
Frameshift Mutation
X Chromosome

Tumor necrosis factor-α and X-linked adrenoleukodystrophy

Mcguinness, M. C., Griffin, D. E., Raymond, G. V., Washington, C. A., Moser, H. W. & Smith, K. D., 1995, In : Journal of Neuroimmunology. 61, 2, p. 161-169 9 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Tumor Necrosis Factor-alpha
X-Linked Genes
Tumor Necrosis Factor Receptors
Genes
1994

Adrenoleukodystrophy update: Genetics and effect of Lorenzo's oil therapy in asymptomatic patients

Moser, H. W., Kok, F., Neumann, S., Borel, J., Bergin, A., Mostafa, S. D., Panoscha, R., Davoli, C. T., Shankroff, J. & Smith, K. D., 1994, In : International Pediatrics. 9, 3, p. 196-204 9 p.

Research output: Contribution to journalArticle

Adrenoleukodystrophy
Mutation
Therapeutics
Gene Deletion
Lorenzo's oil