David R Borchelt

David R Borchelt

Professor, Director SantaFe Health Alzheimer’s Disease Research Center

Department: MD-NEUROSCIENCE-GENERAL
Business Phone: (352) 273-9664
Business Email: drb1@ufl.edu

About David R Borchelt

Dr. Borchelt is a Professor of Neuroscience at the University of Florida College of Medicine and Director of SantaFe Health Alzheimer’s Disease Research Center. He received his PhD in Microbiology/Virology in 1986 from the University of Kentucky and went on to complete a Postdoctoral Fellowship (1986-1989) at the University of California, San Francisco. His laboratory is committed to investigations designed to elucidate the molecular processes by which specific mutant proteins cause disease. This work involves the use of transgenic mouse models, knockout mice, and cell culture systems to examine the effect of mutations on the function and biology of the mutated proteins. Collectively, these approaches provide insight into the molecular mechanisms of disease and have the potential to identify new therapeutic strategies for these disorders.

Research Profile

Dr. Borchelt’s research is focused on defining the basic molecular events that underlie human neurodegenerative diseases, and using this information to develop new therapeutic strategies. To accomplish these goals, his lab uses a variety of model systems to understand how mutations in specific genes cause diseases such as amyotrophic lateral sclerosis (ALS) and Alzheimer’s disease. As they have begun to decipher the mechanisms of these diseases, his lab has found common features that implicate common pathways. One of the common features of these disorders is the presence of pathological structures in brain or spinal cord that are composed of misfolded proteins. Recent studies have revealed that the misfolded pathologic proteins in ALS and Alzheimer’s disease can exhibit prion-like properties to mediate the spread of pathology throughout the brain or spinal cord. His lab is currently focused on understanding the mechanisms by which pathology seems to spread in the nervous system and identify therapies to slow this spread.

Publications

2020
Diversity in Aβ deposit morphology and secondary proteome insolubility across models of Alzheimer-type amyloidosis.
Acta neuropathologica communications. 8(1) [DOI] 10.1186/s40478-020-00911-y. [PMID] 32252825.
2020
Tryptophan residue 32 in human Cu-Zn superoxide dismutase modulates prion-like propagation and strain selection.
PloS one. 15(1) [DOI] 10.1371/journal.pone.0227655. [PMID] 31999698.
2020
Therapeutic approaches targeting Apolipoprotein E function in Alzheimer’s disease.
Molecular neurodegeneration. 15(1) [DOI] 10.1186/s13024-020-0358-9. [PMID] 32005122.
2020
Intracerebral Expression of AAV-APOE4 Is Not Sufficient to Alter Tau Burden in Two Distinct Models of Tauopathy.
Molecular neurobiology. 57(4):1986-2001 [DOI] 10.1007/s12035-019-01859-4. [PMID] 31903524.
2020
Phenotypic diversity in ALS and the role of poly-conformational protein misfolding.
Acta neuropathologica. [DOI] 10.1007/s00401-020-02222-x. [PMID] 32930869.
2020
Subcellular diversion of cholesterol by gain- and loss-of-function mutations in PMP22.
Glia. 68(11):2300-2315 [DOI] 10.1002/glia.23840. [PMID] 32511821.
2019
Aberrant accrual of BIN1 near Alzheimer’s disease amyloid deposits in transgenic models.
Brain pathology (Zurich, Switzerland). 29(4):485-501 [DOI] 10.1111/bpa.12687. [PMID] 30506549.
2019
ALS-Linked SOD1 Mutants Enhance Neurite Outgrowth and Branching in Adult Motor Neurons.
iScience. 11:294-304 [DOI] 10.1016/j.isci.2018.12.026. [PMID] 30639851.
2019
ALS-Linked SOD1 Mutants Enhance Neurite Outgrowth and Branching in Adult Motor Neurons.
iScience. 19:448-449 [DOI] 10.1016/j.isci.2019.08.004. [PMID] 31425915.
2019
Comparative analyses of the in vivo induction and transmission of α-synuclein pathology in transgenic mice by MSA brain lysate and recombinant α-synuclein fibrils.
Acta neuropathologica communications. 7(1) [DOI] 10.1186/s40478-019-0733-3. [PMID] 31109378.
2019
Experimental Mutations in Superoxide Dismutase 1 Provide Insight into Potential Mechanisms Involved in Aberrant Aggregation in Familial Amyotrophic Lateral Sclerosis.
G3 (Bethesda, Md.). 9(3):719-728 [DOI] 10.1534/g3.118.200787. [PMID] 30622123.
2019
N-terminal sequences in matrin 3 mediate phase separation into droplet-like structures that recruit TDP43 variants lacking RNA binding elements.
Laboratory investigation; a journal of technical methods and pathology. 99(7):1030-1040 [DOI] 10.1038/s41374-019-0260-7. [PMID] 31019288.
2018
Targeting the Neuromuscular Junction in ALS.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics. 15(3):713-714 [DOI] 10.1007/s13311-018-0647-y. [PMID] 30006767.
2018
Targeting the accomplice to thwart the culprit: a new target for the prevention of amyloid deposition.
The Journal of clinical investigation. 128(5):1734-1736 [DOI] 10.1172/JCI120414. [PMID] 29600962.
2018
Short Aβ peptides attenuate Aβ42 toxicity in vivo.
The Journal of experimental medicine. 215(1):283-301 [DOI] 10.1084/jem.20170600. [PMID] 29208777.
2018
Prion-like Spreading in Tauopathies.
Biological psychiatry. 83(4):337-346 [DOI] 10.1016/j.biopsych.2017.04.003. [PMID] 28506438.
2018
Loss of charge mutations in solvent exposed Lys residues of superoxide dismutase 1 do not induce inclusion formation in cultured cell models.
PloS one. 13(11) [DOI] 10.1371/journal.pone.0206751. [PMID] 30399166.
2018
Differential induction of mutant SOD1 misfolding and aggregation by tau and α-synuclein pathology.
Molecular neurodegeneration. 13(1) [DOI] 10.1186/s13024-018-0253-9. [PMID] 29776378.
2018
Characterization of gene regulation and protein interaction networks for Matrin 3 encoding mutations linked to amyotrophic lateral sclerosis and myopathy.
Scientific reports. 8(1) [DOI] 10.1038/s41598-018-21371-4. [PMID] 29511296.
2018
Changes in proteome solubility indicate widespread proteostatic disruption in mouse models of neurodegenerative disease.
Acta neuropathologica. 136(6):919-938 [DOI] 10.1007/s00401-018-1895-y. [PMID] 30140941.
2018
Analysis of spinal and muscle pathology in transgenic mice overexpressing wild-type and ALS-linked mutant MATR3.
Acta neuropathologica communications. 6(1) [DOI] 10.1186/s40478-018-0631-0. [PMID] 30563574.
2017
Quantitative Comparison of Dense-Core Amyloid Plaque Accumulation in Amyloid-β Protein Precursor Transgenic Mice.
Journal of Alzheimer's disease : JAD. 56(2):743-761 [DOI] 10.3233/JAD-161027. [PMID] 28059792.
2017
Relationship between mutant Cu/Zn superoxide dismutase 1 maturation and inclusion formation in cell models.
Journal of neurochemistry. 140(1):140-150 [DOI] 10.1111/jnc.13864. [PMID] 27727458.
2017
Retraction Note: Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype.
Acta neuropathologica communications. 5(1) [DOI] 10.1186/s40478-017-0502-0. [PMID] 29237481.
2016
Vulnerability of newly synthesized proteins to proteostasis stress.
Journal of cell science. 129(9):1892-901 [DOI] 10.1242/jcs.176479. [PMID] 27026526.
2016
Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype.
Acta neuropathologica communications. 4(1) [DOI] 10.1186/s40478-016-0393-5. [PMID] 27863507.
2016
Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways.
Acta neuropathologica. 131(1):103-14 [DOI] 10.1007/s00401-015-1514-0. [PMID] 26650262.
2016
Non-prion-type transmission in A53T α-synuclein transgenic mice: a normal component of spinal homogenates from naïve non-transgenic mice induces robust α-synuclein pathology.
Acta neuropathologica. 131(1):151-4 [DOI] 10.1007/s00401-015-1505-1. [PMID] 26541429.
2016
Sex-related dimorphism in dentate gyrus atrophy and behavioral phenotypes in an inducible tTa:APPsi transgenic model of Alzheimer’s disease.
Neurobiology of disease. 96:171-185 [DOI] 10.1016/j.nbd.2016.08.009. [PMID] 27569580.
2016
Generation of a new transgenic mouse model for assessment of tau gene silencing therapies.
Alzheimer's research & therapy. 8 [DOI] 10.1186/s13195-016-0202-1. [PMID] 27593210.
2016
Distinct conformers of transmissible misfolded SOD1 distinguish human SOD1-FALS from other forms of familial and sporadic ALS.
Acta neuropathologica. 132(6):827-840 [PMID] 27704280.
View on: PubMed
2016
C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD.
Neuron. 90(3):521-34 [DOI] 10.1016/j.neuron.2016.04.005. [PMID] 27112499.
2016
Heterogeneity of Matrin 3 in the developing and aging murine central nervous system.
The Journal of comparative neurology. 524(14):2740-52 [DOI] 10.1002/cne.23986. [PMID] 26878116.
2015
Widespread and efficient transduction of spinal cord and brain following neonatal AAV injection and potential disease modifying effect in ALS mice.
Molecular therapy : the journal of the American Society of Gene Therapy. 23(1):53-62 [DOI] 10.1038/mt.2014.180. [PMID] 25228069.
2015
Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation.
Journal of neurochemistry. 133(3):452-64 [DOI] 10.1111/jnc.13022. [PMID] 25557022.
2015
RAN Translation in Huntington Disease.
Neuron. 88(4):667-77 [DOI] 10.1016/j.neuron.2015.10.038. [PMID] 26590344.
2015
Subcellular Localization of Matrin 3 Containing Mutations Associated with ALS and Distal Myopathy.
PloS one. 10(11) [DOI] 10.1371/journal.pone.0142144. [PMID] 26528920.
2015
Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice.
Human molecular genetics. 24(4):1019-35 [DOI] 10.1093/hmg/ddu517. [PMID] 25305079.
2015
Characterization of Protein Structural Changes in Living Cells Using Time-Lapsed FTIR Imaging.
Analytical chemistry. 87(12):6025-31 [DOI] 10.1021/acs.analchem.5b00371. [PMID] 25965274.
2015
Behavioral abnormalities in APPSwe/PS1dE9 mouse model of AD-like pathology: comparative analysis across multiple behavioral domains.
Neurobiology of aging. 36(9):2519-32 [DOI] 10.1016/j.neurobiolaging.2015.05.010. [PMID] 26089165.
2015
Murine Aβ over-production produces diffuse and compact Alzheimer-type amyloid deposits.
Acta neuropathologica communications. 3 [DOI] 10.1186/s40478-015-0252-9. [PMID] 26566997.
2014
Gabriele Schilling (September 5, 1968-July 4, 2014).
Journal of Huntington's disease. 3(3):225-7 [DOI] 10.3233/JHD-149005. [PMID] 25300326.
2014
Regenerative medicine in Alzheimer’s disease.
Translational research : the journal of laboratory and clinical medicine. 163(4):432-8 [DOI] 10.1016/j.trsl.2013.11.001. [PMID] 24286919.
2014
Metal-deficient aggregates and diminished copper found in cells expressing SOD1 mutations that cause ALS.
Frontiers in aging neuroscience. 6 [DOI] 10.3389/fnagi.2014.00110. [PMID] 24982630.
2014
Intramuscular injection of α-synuclein induces CNS α-synuclein pathology and a rapid-onset motor phenotype in transgenic mice.
Proceedings of the National Academy of Sciences of the United States of America. 111(29):10732-7 [DOI] 10.1073/pnas.1321785111. [PMID] 25002524.
2014
Experimental transmissibility of mutant SOD1 motor neuron disease.
Acta neuropathologica. 128(6):791-803 [DOI] 10.1007/s00401-014-1342-7. [PMID] 25262000.
2014
Differences in memory development among C57BL/6NCrl, 129S2/SvPasCrl, and FVB/NCrl mice after delay and trace fear conditioning.
Comparative medicine. 64(1):4-12 [PMID] 24672832.
View on: PubMed
2014
Distinctive features of the D101N and D101G variants of superoxide dismutase 1; two mutations that produce rapidly progressing motor neuron disease.
Journal of neurochemistry. 128(2):305-14 [DOI] 10.1111/jnc.12451. [PMID] 24032979.
2014
Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases.
Acta neuropathologica communications. 2 [DOI] 10.1186/2051-5960-2-55. [PMID] 24887207.
2014
Analysis of mutant SOD1 electrophoretic mobility by Blue Native gel electrophoresis; evidence for soluble multimeric assemblies.
PloS one. 9(8) [DOI] 10.1371/journal.pone.0104583. [PMID] 25121776.
2014
Experimental mutagenesis of huntingtin to map cleavage sites: different outcomes in cell and mouse models.
Journal of Huntington's disease. 3(1):73-86 [DOI] 10.3233/JHD-130087. [PMID] 25062766.
2013
Unbiased screen reveals ubiquilin-1 and -2 highly associated with huntingtin inclusions.
Brain research. 1524:62-73 [DOI] 10.1016/j.brainres.2013.06.006. [PMID] 23774650.
2013
Thinking laterally about neurodegenerative proteinopathies.
The Journal of clinical investigation. 123(5):1847-55 [DOI] 10.1172/JCI66029. [PMID] 23635781.
2013
Structural similarity of wild-type and ALS-mutant superoxide dismutase-1 fibrils using limited proteolysis and atomic force microscopy.
Proceedings of the National Academy of Sciences of the United States of America. 110(27):10934-9 [DOI] 10.1073/pnas.1309613110. [PMID] 23781106.
2013
Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy.
Acta neuropathologica. 126(1):39-50 [DOI] 10.1007/s00401-013-1123-8. [PMID] 23666556.
2013
Normal cognition in transgenic BRI2-Aβ mice.
Molecular neurodegeneration. 8 [DOI] 10.1186/1750-1326-8-15. [PMID] 23663320.
2013
Reversible pathologic and cognitive phenotypes in an inducible model of Alzheimer-amyloidosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience. 33(9):3765-79 [DOI] 10.1523/JNEUROSCI.4251-12.2013. [PMID] 23447589.
2013
Cytosolic proteins lose solubility as amyloid deposits in a transgenic mouse model of Alzheimer-type amyloidosis.
Human molecular genetics. 22(14):2765-74 [DOI] 10.1093/hmg/ddt121. [PMID] 23512986.
2013
An analysis of interactions between fluorescently-tagged mutant and wild-type SOD1 in intracellular inclusions.
PloS one. 8(12) [DOI] 10.1371/journal.pone.0083981. [PMID] 24391857.
2013
Capsid serotype and timing of injection determines AAV transduction in the neonatal mice brain.
PloS one. 8(6) [DOI] 10.1371/journal.pone.0067680. [PMID] 23825679.
2013
Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1.
Molecular neurodegeneration. 8 [DOI] 10.1186/1750-1326-8-46. [PMID] 24341866.
2013
Comment on “ApoE-directed therapeutics rapidly clear β-amyloid and reverse deficits in AD mouse models”.
Science (New York, N.Y.). 340(6135):924-d [DOI] 10.1126/science.1234089. [PMID] 23704553.
2012
A novel variant of human superoxide dismutase 1 harboring amyotrophic lateral sclerosis-associated and experimental mutations in metal-binding residues and free cysteines lacks toxicity in vivo.
Journal of neurochemistry. 121(3):475-85 [DOI] 10.1111/j.1471-4159.2012.07690.x. [PMID] 22332887.
2012
A preclinical assessment of neural stem cells as delivery vehicles for anti-amyloid therapeutics.
PloS one. 7(4) [DOI] 10.1371/journal.pone.0034097. [PMID] 22496779.
2012
Abnormal SDS-PAGE migration of cytosolic proteins can identify domains and mechanisms that control surfactant binding.
Protein science : a publication of the Protein Society. 21(8):1197-209 [DOI] 10.1002/pro.2107. [PMID] 22692797.
2012
Analysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model.
PloS one. 7(12) [DOI] 10.1371/journal.pone.0050750. [PMID] 23236391.
2012
Ex vivo cultures of microglia from young and aged rodent brain reveal age-related changes in microglial function.
Neurobiology of aging. 33(1):195.e1-12 [DOI] 10.1016/j.neurobiolaging.2010.05.008. [PMID] 20580465.
2012
Identification of proteins sensitive to thermal stress in human neuroblastoma and glioma cell lines.
PloS one. 7(11) [DOI] 10.1371/journal.pone.0049021. [PMID] 23145051.
2012
Reduction of low-density lipoprotein receptor-related protein (LRP1) in hippocampal neurons does not proportionately reduce, or otherwise alter, amyloid deposition in APPswe/PS1dE9 transgenic mice.
Alzheimer's research & therapy. 4(2) [DOI] 10.1186/alzrt110. [PMID] 22537779.
2012
Role of disulfide cross-linking of mutant SOD1 in the formation of inclusion-body-like structures.
PloS one. 7(10) [DOI] 10.1371/journal.pone.0047838. [PMID] 23118898.
2011
Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative.
Human molecular genetics. 20(14):2770-82 [DOI] 10.1093/hmg/ddr176. [PMID] 21515588.
2011
Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment.
Human molecular genetics. 20(8):1633-42 [DOI] 10.1093/hmg/ddr040. [PMID] 21307087.
2011
Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states.
Molecular neurodegeneration. 6 [DOI] 10.1186/1750-1326-6-77. [PMID] 22094223.
2011
Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice.
The Journal of biological chemistry. 286(4):2795-806 [DOI] 10.1074/jbc.M110.186999. [PMID] 21068388.
2011
Cellular fusion for gene delivery to SCA1 affected Purkinje neurons.
Molecular and cellular neurosciences. 47(1):61-70 [DOI] 10.1016/j.mcn.2011.03.003. [PMID] 21420496.
2011
Passive (amyloid-β) immunotherapy attenuates monoaminergic axonal degeneration in the AβPPswe/PS1dE9 mice.
Journal of Alzheimer's disease : JAD. 23(2):271-9 [DOI] 10.3233/JAD-2010-101602. [PMID] 20966549.
2010
Aggregation modulating elements in mutant human superoxide dismutase 1.
Archives of biochemistry and biophysics. 503(2):175-82 [DOI] 10.1016/j.abb.2010.07.027. [PMID] 20682279.
2010
An examination of alpha B-crystallin as a modifier of SOD1 aggregate pathology and toxicity in models of familial amyotrophic lateral sclerosis.
Journal of neurochemistry. 113(5):1092-100 [DOI] 10.1111/j.1471-4159.2010.06572.x. [PMID] 20067574.
2010
An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1.
Human molecular genetics. 19(24):4774-89 [DOI] 10.1093/hmg/ddq408. [PMID] 20871097.
2010
Analysis of chaperone mRNA expression in the adult mouse brain by meta analysis of the Allen Brain Atlas.
PloS one. 5(10) [DOI] 10.1371/journal.pone.0013675. [PMID] 21060842.
2010
Partial depletion of CREB-binding protein reduces life expectancy in a mouse model of Huntington disease.
Journal of neuropathology and experimental neurology. 69(4):396-404 [DOI] 10.1097/NEN.0b013e3181d6c436. [PMID] 20448484.
2010
Synphilin-1 attenuates neuronal degeneration in the A53T alpha-synuclein transgenic mouse model.
Human molecular genetics. 19(11):2087-98 [DOI] 10.1093/hmg/ddq086. [PMID] 20185556.
2009
Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS.
Proceedings of the National Academy of Sciences of the United States of America. 106(19):7774-9 [DOI] 10.1073/pnas.0902505106. [PMID] 19416874.
2009
Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease.
Human molecular genetics. 18(17):3217-26 [DOI] 10.1093/hmg/ddp260. [PMID] 19483195.
2009
Variation in Aggregate Levels of Disease-Associated Variants of Sod1: Correlation To Human Disease
Journal of Neurochemistry. 108
2009
Structural and biophysical properties of the pathogenic SOD1 variant H46R/H48Q.
Biochemistry. 48(15):3436-47 [DOI] 10.1021/bi8021735. [PMID] 19227972.
2009
Protein aggregate characterization in models of neurodegenerative disease.
Methods in molecular biology (Clifton, N.J.). 566:85-91 [DOI] 10.1007/978-1-59745-562-6_6. [PMID] 20058166.
2009
Immunoreactivity of the phosphorylated axonal neurofilament H subunit (pNF-H) in blood of ALS model rodents and ALS patients: evaluation of blood pNF-H as a potential ALS biomarker.
Journal of neurochemistry. 111(5):1182-91 [DOI] 10.1111/j.1471-4159.2009.06386.x. [PMID] 19765193.
2009
Immunotherapy Attenuates Monoaminergic Neurodegeneration in Transgenic Mice
Journal of the American Geriatrics Society. 57:S11-S12
2009
Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme.
Journal of neurochemistry. 108(4):1009-18 [DOI] 10.1111/j.1471-4159.2008.05839.x. [PMID] 19077113.
2009
Amyloid precursor protein increases cortical neuron size in transgenic mice.
Neurobiology of aging. 30(8):1238-44 [DOI] 10.1016/j.neurobiolaging.2007.12.024. [PMID] 18304698.
2009
Expression of Human Wt Sod1 Slows Aggregation of Mutant Sod1, But Eventually Co-Aggregates With the Mutant Protein
Journal of Neurochemistry. 108
2009
Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis.
Experimental biology and medicine (Maywood, N.J.). 234(10):1140-54 [DOI] 10.3181/0903-MR-104. [PMID] 19596823.
2008
A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis.
The Journal of biological chemistry. 283(20):13528-37 [DOI] 10.1074/jbc.M800564200. [PMID] 18316367.
2008
Receptor-associated protein (RAP) plays a central role in modulating Abeta deposition in APP/PS1 transgenic mice.
PloS one. 3(9) [DOI] 10.1371/journal.pone.0003159. [PMID] 18776935.
2008
Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS.
PloS one. 3(8) [DOI] 10.1371/journal.pone.0002849. [PMID] 18682740.
2008
Limited clearance of pre-existing amyloid plaques after intracerebral injection of Abeta antibodies in two mouse models of Alzheimer disease.
Journal of neuropathology and experimental neurology. 67(1):30-40 [PMID] 18091561.
View on: PubMed
2008
Studies of Blood and Csf Biomarkers of Cns Damage and Disease
Journal of Neurotrauma. 25
2008
Detergent-insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1.
The Journal of biological chemistry. 283(13):8340-50 [DOI] 10.1074/jbc.M707751200. [PMID] 18192269.
2008
Amyloid pathology is associated with progressive monoaminergic neurodegeneration in a transgenic mouse model of Alzheimer’s disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience. 28(51):13805-14 [DOI] 10.1523/JNEUROSCI.4218-08.2008. [PMID] 19091971.
2008
Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases.
Neurobiology of aging. 29(4):586-97 [PMID] 17316906.
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2008
MnSOD deficiency has a differential effect on disease progression in two different ALS mutant mouse models.
Muscle & nerve. 38(3):1173-83 [DOI] 10.1002/mus.21049. [PMID] 18720509.
2007
Rodent A beta modulates the solubility and distribution of amyloid deposits in transgenic mice.
The Journal of biological chemistry. 282(31):22707-20 [PMID] 17556372.
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2007
Alzheimer’s-type amyloidosis in transgenic mice impairs survival of newborn neurons derived from adult hippocampal neurogenesis.
The Journal of neuroscience : the official journal of the Society for Neuroscience. 27(25):6771-80 [PMID] 17581964.
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2007
Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models.
Journal of neuropathology and experimental neurology. 66(4):313-20 [PMID] 17413322.
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2007
Disease-associated mutations at copper ligand histidine residues of superoxide dismutase 1 diminish the binding of copper and compromise dimer stability.
The Journal of biological chemistry. 282(1):345-52 [PMID] 17092942.
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2007
Human health risk assessment for aluminium, aluminium oxide, and aluminium hydroxide.
Journal of toxicology and environmental health. Part B, Critical reviews. 10 Suppl 1:1-269 [PMID] 18085482.
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2007
Investigation of RNA interference to suppress expression of full-length and fragment human huntingtin.
Neuromolecular medicine. 9(2):145-55 [PMID] 17627034.
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2007
N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington’s disease.
Cell cycle (Georgetown, Tex.). 6(23):2970-81 [PMID] 18156806.
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2006
Amyotrophic lateral sclerosis–are microglia killing motor neurons?
The New England journal of medicine. 355(15):1611-3 [PMID] 17035656.
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2006
Papillomavirus-like particles are an effective platform for amyloid-beta immunization in rabbits and transgenic mice.
Journal of immunology (Baltimore, Md. : 1950). 177(4):2662-2670 [PMID] 16888028.
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2006
Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin.
Neurobiology of disease. 21(2):381-91 [PMID] 16150600.
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2006
Mapping superoxide dismutase 1 domains of non-native interaction: roles of intra- and intermolecular disulfide bonding in aggregation.
Journal of neurochemistry. 96(5):1277-88 [PMID] 16441516.
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2005
BACE1, a major determinant of selective vulnerability of the brain to amyloid-beta amyloidogenesis, is essential for cognitive, emotional, and synaptic functions.
The Journal of neuroscience : the official journal of the Society for Neuroscience. 25(50):11693-709 [PMID] 16354928.
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2005
Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA.
Neurobiology of disease. 20(3):943-52 [PMID] 16046140.
View on: PubMed
2005
Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington’s disease.
Journal of neurochemistry. 95(2):553-62 [PMID] 16135087.
View on: PubMed
2005
Environmental enrichment mitigates cognitive deficits in a mouse model of Alzheimer’s disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience. 25(21):5217-24 [PMID] 15917461.
View on: PubMed
2005
Episodic-like memory deficits in the APPswe/PS1dE9 mouse model of Alzheimer’s disease: relationships to beta-amyloid deposition and neurotransmitter abnormalities.
Neurobiology of disease. 18(3):602-17 [PMID] 15755686.
View on: PubMed
2005
Persistent amyloidosis following suppression of Abeta production in a transgenic model of Alzheimer disease.
PLoS medicine. 2(12) [PMID] 16279840.
View on: PubMed
2005
Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: alphaB-crystallin modulates aggregation.
Human molecular genetics. 14(16):2335-47 [PMID] 16000321.
View on: PubMed
2004
Nuclear-targeting of mutant huntingtin fragments produces Huntington’s disease-like phenotypes in transgenic mice.
Human molecular genetics. 13(15):1599-610 [PMID] 15190011.
View on: PubMed
2004
Mutant presenilins specifically elevate the levels of the 42 residue beta-amyloid peptide in vivo: evidence for augmentation of a 42-specific gamma secretase.
Human molecular genetics. 13(2):159-70 [PMID] 14645205.
View on: PubMed
2004
Environmental, pharmacological, and genetic modulation of the HD phenotype in transgenic mice.
Experimental neurology. 187(1):137-49 [PMID] 15081595.
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2004
APP processing and amyloid deposition in mice haplo-insufficient for presenilin 1.
Neurobiology of aging. 25(7):885-92 [PMID] 15212842.
View on: PubMed
2003
APP processing and synaptic function.
Neuron. 37(6):925-37 [PMID] 12670422.
View on: PubMed
2003
Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature.
Human molecular genetics. 12(21):2753-64 [PMID] 12966034.
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2003
Environmental enrichment exacerbates amyloid plaque formation in a transgenic mouse model of Alzheimer disease.
Journal of neuropathology and experimental neurology. 62(12):1220-7 [PMID] 14692698.
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2003
Lipopolysaccharide-induced-neuroinflammation increases intracellular accumulation of amyloid precursor protein and amyloid beta peptide in APPswe transgenic mice.
Neurobiology of disease. 14(1):133-45 [PMID] 13678674.
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2003
Normal cognitive behavior in two distinct congenic lines of transgenic mice hyperexpressing mutant APP SWE.
Neurobiology of disease. 12(3):194-211 [PMID] 12742740.
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2002
Transgenic mouse models of neurodegenerative disease: opportunities for therapeutic development.
Current neurology and neuroscience reports. 2(5):457-64 [PMID] 12169227.
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2002
Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington’s disease mouse models reveal context-independent effects.
Human molecular genetics. 11(17):1927-37 [PMID] 12165555.
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2002
High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation.
Neurobiology of disease. 9(2):139-48 [PMID] 11895367.
View on: PubMed
2002
Genetically engineered mouse models of neurodegenerative diseases.
Nature neuroscience. 5(7):633-9 [PMID] 12085093.
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2002
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site.
Neurobiology of disease. 10(2):128-38 [PMID] 12127151.
View on: PubMed
2002
Rapid detection of protein aggregates in the brains of Alzheimer patients and transgenic mouse models of amyloidosis.
Alzheimer disease and associated disorders. 16(3):191-5 [PMID] 12218651.
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2002
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice.
Human molecular genetics. 11(6):633-40 [PMID] 11912178.
View on: PubMed
2002
Cyclooxygenase (COX)-2 and cell cycle activity in a transgenic mouse model of Alzheimer’s disease neuropathology.
Neurobiology of aging. 23(3):327-34 [PMID] 11959394.
View on: PubMed
2002
Accumulation of proteolytic fragments of mutant presenilin 1 and accelerated amyloid deposition are co-regulated in transgenic mice.
Neurobiology of aging. 23(2):171-7 [PMID] 11804700.
View on: PubMed
2002
Abeta deposition does not cause the aggregation of endogenous tau in transgenic mice.
Alzheimer disease and associated disorders. 16(3):196-201 [PMID] 12218652.
View on: PubMed
2002
Abeta deposition is essential to AD neuropathology.
Journal of Alzheimer's disease : JAD. 4(3):133-8 [PMID] 12226531.
View on: PubMed
2002
Expression of stabilized beta-catenin in differentiated neurons of transgenic mice does not result in tumor formation.
BMC cancer. 2 [PMID] 12460454.
View on: PubMed
2001
Hyper-expression of human apolipoprotein E4 in astroglia and neurons does not enhance amyloid deposition in transgenic mice.
Human molecular genetics. 10(22):2525-37 [PMID] 11709540.
View on: PubMed
2001
Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics.
Journal of molecular neuroscience : MN. 17(2):233-57 [PMID] 11816796.
View on: PubMed
2001
Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA.
Neurobiology of disease. 8(3):405-18 [PMID] 11442350.
View on: PubMed
2001
Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington’s disease.
Annals of neurology. 50(1):112-7 [PMID] 11456300.
View on: PubMed
2001
Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington’s disease transgenic mouse model.
Neuroscience letters. 315(3):149-53 [PMID] 11716985.
View on: PubMed
2001
Co-expression of multiple transgenes in mouse CNS: a comparison of strategies.
Biomolecular engineering. 17(6):157-65 [PMID] 11337275.
View on: PubMed
2001
beta-Amyloid peptide vaccination results in marked changes in serum and brain Abeta levels in APPswe/PS1DeltaE9 mice, as detected by SELDI-TOF-based ProteinChip technology.
DNA and cell biology. 20(11):713-21 [PMID] 11788049.
View on: PubMed
2001
BACE1 is the major beta-secretase for generation of Abeta peptides by neurons.
Nature neuroscience. 4(3):233-4 [PMID] 11224536.
View on: PubMed
2001
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington’s disease.
Neurobiology of disease. 8(3):479-91 [PMID] 11447996.
View on: PubMed
2000
The value of transgenic models for the study of neurodegenerative diseases.
Annals of the New York Academy of Sciences. 920:179-91 [PMID] 11193148.
View on: PubMed
2000
Enhanced synaptic potentiation in transgenic mice expressing presenilin 1 familial Alzheimer’s disease mutation is normalized with a benzodiazepine.
Neurobiology of disease. 7(1):54-63 [PMID] 10671322.
View on: PubMed
2000
Decreased expression of striatal signaling genes in a mouse model of Huntington’s disease.
Human molecular genetics. 9(9):1259-71 [PMID] 10814708.
View on: PubMed
2000
Amyloid precursor proteins inhibit heme oxygenase activity and augment neurotoxicity in Alzheimer’s disease.
Neuron. 28(2):461-73 [PMID] 11144356.
View on: PubMed
1999
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.
Human molecular genetics. 8(3):397-407 [PMID] 9949199.
View on: PubMed
1999
Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.
Neuron. 24(1):275-86 [PMID] 10677044.
View on: PubMed
1999
Polyglutamine pathogenesis.
Philosophical transactions of the Royal Society of London. Series B, Biological sciences. 354(1386):1005-11 [PMID] 10434299.
View on: PubMed
1999
SOD1 rescues cerebral endothelial dysfunction in mice overexpressing amyloid precursor protein.
Nature neuroscience. 2(2):157-61 [PMID] 10195200.
View on: PubMed
1999
Synaptic transmission and hippocampal long-term potentiation in transgenic mice expressing FAD-linked presenilin 1.
Neurobiology of disease. 6(1):56-62 [PMID] 10078973.
View on: PubMed
1999
Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds.
Human molecular genetics. 8(8):1451-60 [PMID] 10400992.
View on: PubMed
1998
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.
Human molecular genetics. 7(5):783-90 [PMID] 9536081.
View on: PubMed
1998
Transgenic mouse models of Alzheimer’s disease and amyotrophic lateral sclerosis.
Brain pathology (Zurich, Switzerland). 8(4):735-57 [PMID] 9804381.
View on: PubMed
1998
Stable association of presenilin derivatives and absence of presenilin interactions with APP.
Neurobiology of disease. 4(6):438-53 [PMID] 9666482.
View on: PubMed
1998
Metabolism of presenilin 1: influence of presenilin 1 on amyloid precursor protein processing.
Neurobiology of aging. 19(1 Suppl):S15-8 [PMID] 9562461.
View on: PubMed
1998
Inherited neurodegenerative diseases and transgenic models.
Laboratory animal science. 48(6):604-10 [PMID] 10090084.
View on: PubMed
1998
Genetic neurodegenerative diseases: the human illness and transgenic models.
Science (New York, N.Y.). 282(5391):1079-83 [PMID] 9804539.
View on: PubMed
1998
Familial amyotrophic lateral sclerosis and Alzheimer’s disease. Transgenic models.
Advances in experimental medicine and biology. 446:145-59 [PMID] 10079842.
View on: PubMed
1998
Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.
Proceedings of the National Academy of Sciences of the United States of America. 95(26):15763-8 [PMID] 9861044.
View on: PubMed
1998
Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice.
Neurobiology of disease. 5(1):27-35 [PMID] 9702785.
View on: PubMed
1998
Alzheimer disease–when and why?
Nature genetics. 19(4):314-6 [PMID] 9697686.
View on: PubMed
1998
Alzheimer’s disease: genetic studies and transgenic models.
Annual review of genetics. 32:461-93 [PMID] 9928488.
View on: PubMed
1998
Effects of PS1 deficiency on membrane protein trafficking in neurons.
Neuron. 21(5):1213-21 [PMID] 9856475.
View on: PubMed
1998
Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice
Neurobiology of Disease. 5:27-35
1998
An Alzheimer’s disease-linked PS1 variant rescues the developmental abnormalities of PS1-deficient embryos.
Neuron. 20(3):603-9 [PMID] 9539132.
View on: PubMed
1997
Accelerated amyloid deposition in the brains of transgenic mice coexpressing mutant presenilin 1 and amyloid precursor proteins.
Neuron. 19(4):939-45 [PMID] 9354339.
View on: PubMed
1997
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.
Neuron. 18(2):327-38 [PMID] 9052802.
View on: PubMed
1997
Amyotrophic lateral sclerosis and Alzheimer disease. Lessons from model systems.
Revue neurologique. 153(8-9):484-95 [PMID] 9683997.
View on: PubMed
1997
Endoproteolytic processing and stabilization of wild-type and mutant presenilin.
The Journal of biological chemistry. 272(39):24536-41 [PMID] 9305918.
View on: PubMed
1997
Evidence that levels of presenilins (PS1 and PS2) are coordinately regulated by competition for limiting cellular factors.
The Journal of biological chemistry. 272(45):28415-22 [PMID] 9353300.
View on: PubMed
1997
Genetic modification of the phenotypes produced by amyloid precursor protein overexpression in transgenic mice.
Human molecular genetics. 6(11):1951-9 [PMID] 9302276.
View on: PubMed
1997
Hyperaccumulation of FAD-linked presenilin 1 variants in vivo.
Nature medicine. 3(7):756-60 [PMID] 9212102.
View on: PubMed
1996
Transgenic models of neurodegenerative diseases.
Current opinion in neurobiology. 6(5):651-60 [PMID] 8937830.
View on: PubMed
1996
Protein topology of presenilin 1.
Neuron. 17(5):1023-30 [PMID] 8938133.
View on: PubMed
1996
Neurodegenerative diseases and model systems.
Cold Spring Harbor symposia on quantitative biology. 61:725-38 [PMID] 9246498.
View on: PubMed
1996
Motor neuron disease and model systems: aetiologies, mechanisms and therapies.
Ciba Foundation symposium. 196:3-13; discussion 13 [PMID] 8866125.
View on: PubMed
1996
Mechanisms of selective motor neuron death in transgenic mouse models of motor neuron disease.
Neurology. 47(4 Suppl 2):S54-61; discussion S61 [PMID] 8858052.
View on: PubMed
1996
Familial Alzheimer’s disease-linked presenilin 1 variants elevate Abeta1-42/1-40 ratio in vitro and in vivo.
Neuron. 17(5):1005-13 [PMID] 8938131.
View on: PubMed
1996
Inherited neurodegenerative diseases and transgenic models.
Brain pathology (Zurich, Switzerland). 6(4):467-80 [PMID] 8944317.
View on: PubMed
1996
Endoproteolysis of presenilin 1 and accumulation of processed derivatives in vivo.
Neuron. 17(1):181-90 [PMID] 8755489.
View on: PubMed
1996
Loss of functional prion protein: a role in prion disorders?
Chemistry & biology. 3(8):619-21 [PMID] 8807894.
View on: PubMed
1996
A vector for expressing foreign genes in the brains and hearts of transgenic mice.
Genetic analysis : biomolecular engineering. 13(6):159-63 [PMID] 9117892.
View on: PubMed
1995
Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells.
Proceedings of the National Academy of Sciences of the United States of America. 92(7):3024-8 [PMID] 7708768.
View on: PubMed
1995
Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons.
Proceedings of the National Academy of Sciences of the United States of America. 92(4):954-8 [PMID] 7862672.
View on: PubMed
1995
Superoxide dismutase 1 subunits with mutations linked to familial amyotrophic lateral sclerosis do not affect wild-type subunit function.
The Journal of biological chemistry. 270(7):3234-8 [PMID] 7852409.
View on: PubMed
1995
Motor neuron disease caused by mutations in superoxide dismutase 1.
Current opinion in neurology. 8(4):294-301 [PMID] 7582045.
View on: PubMed
1995
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria.
Neuron. 14(6):1105-16 [PMID] 7605627.
View on: PubMed
1995
Age-related CNS disorder and early death in transgenic FVB/N mice overexpressing Alzheimer amyloid precursor proteins.
Neuron. 15(5):1203-18 [PMID] 7576662.
View on: PubMed
1995
Cellular and molecular biology of Alzheimer’s disease and animal models.
Neuroimaging clinics of North America. 5(1):59-68 [PMID] 7743085.
View on: PubMed
1994
A computer-based approach to quality improvement for telephone triage in a community AIDS clinic.
Nursing administration quarterly. 18(2):65-73 [PMID] 8159333.
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1994
Rapid anterograde axonal transport of the cellular prion glycoprotein in the peripheral and central nervous systems.
The Journal of biological chemistry. 269(20):14711-4 [PMID] 7514179.
View on: PubMed
1994
Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity.
Proceedings of the National Academy of Sciences of the United States of America. 91(17):8292-6 [PMID] 8058797.
View on: PubMed
1993
Alzheimer disease and the prion disorders amyloid beta-protein and prion protein amyloidoses.
Proceedings of the National Academy of Sciences of the United States of America. 90(14):6381-4 [PMID] 8101988.
View on: PubMed
1993
Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor
Glycobiology. 3:319-329
1993
Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor.
Glycobiology. 3(4):319-29 [PMID] 7691278.
View on: PubMed
1992
Toxicity of synthetic A beta peptides and modeling of Alzheimer’s disease.
Neurobiology of aging. 13(5):623-5 [PMID] 1461354.
View on: PubMed
1992
Evidence for synthesis of scrapie prion proteins in the endocytic pathway.
The Journal of biological chemistry. 267(23):16188-99 [PMID] 1353761.
View on: PubMed
1992
Synthesis and trafficking of prion proteins in cultured cells.
Molecular biology of the cell. 3(8):851-63 [PMID] 1356522.
View on: PubMed
1992
A needs analysis for computer-based telephone triage in a community AIDS clinic.
Proceedings. Symposium on Computer Applications in Medical Care. 59-63 [PMID] 1482941.
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1992
Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems.
Journal of virology. 66(10):6155-63 [PMID] 1356161.
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1990
Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation.
Proceedings of the National Academy of Sciences of the United States of America. 87(21):8262-6 [PMID] 1978322.
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1990
Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C.
Biochemistry. 29(22):5405-12 [PMID] 1974460.
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1990
Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells.
The Journal of cell biology. 110(3):743-52 [PMID] 1968466.
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1989
Asparagine-linked glycosylation of the scrapie and cellular prion proteins.
Archives of biochemistry and biophysics. 274(1):1-13 [PMID] 2505674.
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1989
Inhibition of avian retrovirus protein synthesis in the presence of host cellular mRNA.
Archives of virology. 107(3-4):261-71 [PMID] 2554857.
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1988
Identification of an initial site of interaction and possible helix destabilizing activity preceding initiation of protein synthesis from retrovirus RNA.
Virus research. 10(2-3):241-8 [PMID] 2842973.
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1988
Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins.
Journal of virology. 62(5):1558-64 [PMID] 3282080.
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1987
Scrapie prion protein contains a phosphatidylinositol glycolipid.
Cell. 51(2):229-40 [PMID] 2444340.
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1985
Influence of base-pairing in the leader region on in vitro translation of Rous sarcoma virus RNA.
Virus research. 3(2):141-51 [PMID] 2998116.
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1982
Participation of 5′-terminal leader sequences in in vitro translation of Rous sarcoma virus RNA.
The Journal of biological chemistry. 257(11):6551-5 [PMID] 6281276.
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Grants

Jan 2020 ACTIVE
Provenance Initiatives Fund: Towards the identification of new therapies for ALS
Role: Principal Investigator
Funding: UF FOU
Sep 2019 ACTIVE
Developing new conditional models to study tauopathy, amyloidosis, and their interaction
Role:
Funding: NATL INST OF HLTH NINDS
Sep 2018 ACTIVE
Prion and non-prion induction mechanisms of alpha-synuclein pathology
Role:
Funding: NATL INST OF HLTH NINDS
Jun 2018 ACTIVE
APOE as a modifier of prion-like spread in dementia
Role: Principal Investigator
Funding: NATL INST OF HLTH NIA
Sep 2017 – May 2018
APOE as a modifier of prion-like spread in dementia
Role: Principal Investigator
Funding: NATL INST OF HLTH NIA
Aug 2017 – Apr 2019
New Drug Discovery Paradigms for Synucleinopathy
Role: Principal Investigator
Funding: LANKENAU INSTITUTE FOR MEDICAL RESEARCH via NATL INST OF HLTH NIA
Dec 2016 – Nov 2019
Is there a form of benign brain amyloidosis in aging?
Role: Principal Investigator
Funding: NATL INST OF HLTH NIA
Aug 2016 – Sep 2019
New mouse models to dissect out the function of the ALS-associated protein Matrin 3
Role:
Funding: AMYOTROPHIC LATERAL SCLEROSIS ASSOC
Aug 2015 ACTIVE
University of Florida – Mt. Sinai Medical Center AD Research Center
Role:
Funding: NATL INST OF HLTH NIA
Jul 2015 ACTIVE
Modeling the progression of SOD1-linked motor neuron disease
Role: Principal Investigator
Funding: NATL INST OF HLTH NINDS
Jun 2015 – Jun 2017
Inhibitors of SOD1 interaction as an approach to slow th
Role: Principal Investigator
Funding: US ARMY MED RES ACQUISITION
Jun 2015 – May 2017
Role Misfolded Wild-Type SOD1 in Cases of Sporadic ALS
Role: Principal Investigator
Funding: JOHNS HOPKINS UNIV
May 2015 ACTIVE
Proteostasis and secondary proteinopathy in AD and FTD
Role: Principal Investigator
Funding: NATL INST OF HLTH NIA
May 2015 – Mar 2018
Seeded transmission of SOD1 misfolding
Role: Principal Investigator
Funding: NATL INST OF HLTH NINDS
Jun 2014 – May 2017
Novel Therapeutics for activation of the antioxidant res
Role: Principal Investigator
Funding: UF DSR OPPORTUNITY FUND
May 2014 – Feb 2017
Seeded models of AD pathology
Role: Principal Investigator
Funding: NATL INST OF HLTH NIA
Jul 2008 ACTIVE
SantaFe HealthCare Alzheimer's Disease Research Center
Role: Principal Investigator
Funding: UF FOU
Jul 2006 ACTIVE
BRAIN AND SPINAL CORD INJURY RESEARCH
Role: Project Manager
Funding: FL DEPT OF HLTH

Teaching Profile

Courses Taught
2009-2013,2015-2020,2019-2020
GMS6750 Molecular Pathobiology of Neural Disease
2009-2010,2019
GMS7794 Neuroscience Seminar
2019
GMS6757 Introduction to Alzheimer’s Disease and Related Dementias: Clinical and Mechanistic Principles
2018
GMS7980 Research for Doctoral Dissertation
2018
GMS7979 Advanced Research
2007-2010,2013
GMS6029 Brain Journal Club

Contact Details

Phones:
Business:
(352) 273-9664
Emails:
Business:
drb1@ufl.edu