David R Borchelt

David R Borchelt,

Professor, Director SantaFe Health Alzheimer’s Disease Research Center

Department: Department of Neuroscience
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.

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
2018,2023-2024
GMS7980 Research for Doctoral Dissertation
2018
GMS7979 Advanced Research
2007-2010,2013-2022,2021-2022
GMS6029 Brain Journal Club
2022-2023
GMS6905 Independent Studies in Medical Sciences
2023
GMS6090 Research in Medical Sciences

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.

Open Researcher and Contributor ID (ORCID)

0000-0002-0813-6979

Publications

2023
Aβ Amyloid Scaffolds the Accumulation of Matrisome and Additional Proteins in Alzheimer’s Disease.
bioRxiv : the preprint server for biology. [DOI] 10.1101/2023.11.29.568318. [PMID] 38076912.
2023
Carboxyl truncation of α-synuclein occurs early and is influenced by human APOE genotype in transgenic mouse models of α-synuclein pathogenesis.
Acta neuropathologica communications. 11(1) [DOI] 10.1186/s40478-023-01623-9. [PMID] 37482615.
2023
Human tauopathy strains defined by phosphorylation in R1-R2 repeat domains of tau.
Acta neuropathologica communications. 11(1) [DOI] 10.1186/s40478-023-01664-0. [PMID] 37891635.
2023
Humanized APOE genotypes influence lifespan independently of tau aggregation in the P301S mouse model of tauopathy.
Acta neuropathologica communications. 11(1) [DOI] 10.1186/s40478-023-01581-2. [PMID] 37337279.
2023
Multiple Factors Influence the Incubation Period of ALS Prion-like Transmission in SOD1 Transgenic Mice
Viruses. 15(9) [DOI] 10.3390/v15091819. [PMID] 37766226.
2022
Blood-based biomarkers of inflammation in amyotrophic lateral sclerosis.
Molecular neurodegeneration. 17(1) [DOI] 10.1186/s13024-022-00515-1. [PMID] 35073950.
2022
Impact of APOE genotype on prion-type propagation of tauopathy.
Acta neuropathologica communications. 10(1) [DOI] 10.1186/s40478-022-01359-y. [PMID] 35440098.
2022
Modeling the Competition between Misfolded Aβ Conformers That Produce Distinct Types of Amyloid Pathology in Alzheimer’s Disease
Biomolecules. 12(7) [DOI] 10.3390/biom12070886. [PMID] 35883442.
2022
Pathogenic tau recruits wild-type tau into brain inclusions and induces gut degeneration in transgenic SPAM mice.
Communications biology. 5(1) [DOI] 10.1038/s42003-022-03373-1. [PMID] 35550593.
2022
Soluble brain homogenates from diverse human and mouse sources preferentially seed diffuse Aβ plaque pathology when injected into newborn mouse hosts.
Free neuropathology. 3(9) [DOI] 10.17879/freeneuropathology-2022-3766. [PMID] 35494163.
2022
TAPPing into the potential of inducible tau/APP transgenic mice.
Neuropathology and applied neurobiology. 48(3) [DOI] 10.1111/nan.12791. [PMID] 35067965.
2021
Astrocytic apoE4 and tau: Deadly combination for neurons.
Cell reports. Medicine. 2(6) [DOI] 10.1016/j.xcrm.2021.100316. [PMID] 34195681.
2021
Author Correction: IL-10 based immunomodulation initiated at birth extends lifespan in a familial mouse model of amyotrophic lateral sclerosis.
Scientific reports. 11(1) [DOI] 10.1038/s41598-021-83092-5. [PMID] 33536539.
2021
Building a Case for Withaferin A as a Treatment for FTD/ALS Syndromes
Neurotherapeutics. 18(1):284-285 [DOI] 10.1007/s13311-020-00991-7. [PMID] 33398802.
2021
Collusion of α-Synuclein and Aβ aggravating co-morbidities in a novel prion-type mouse model.
Molecular neurodegeneration. 16(1) [DOI] 10.1186/s13024-021-00486-9. [PMID] 34503546.
2021
Il-10 signaling reduces survival in mouse models of synucleinopathy.
NPJ Parkinson's disease. 7(1) [DOI] 10.1038/s41531-021-00169-8. [PMID] 33741985.
2021
Novel SOD1 monoclonal antibodies against the electrostatic loop preferentially detect misfolded SOD1 aggregates
Neuroscience Letters. 742 [DOI] 10.1016/j.neulet.2020.135553. [PMID] 33346076.
2021
Phenotypic diversity in ALS and the role of poly-conformational protein misfolding.
Acta neuropathologica. 142(1):41-55 [DOI] 10.1007/s00401-020-02222-x. [PMID] 32930869.
2021
Reactive astrocytes as treatment targets in Alzheimer’s disease—Systematic review of studies using the APPswePS1dE9 mouse model
Glia. 69(8):1852-1881 [DOI] 10.1002/glia.23981. [PMID] 33634529.
2021
Remodeling Alzheimer-amyloidosis models by seeding.
Molecular neurodegeneration. 16(1) [DOI] 10.1186/s13024-021-00429-4. [PMID] 33588898.
2021
Supercharging Prions via Amyloid‐Selective Lysine Acetylation
Angewandte Chemie. 133(27):15196-15206 [DOI] 10.1002/ange.202103548.
2021
Supercharging Prions via Amyloid‐Selective Lysine Acetylation
Angewandte Chemie International Edition. 60(27):15069-15079 [DOI] 10.1002/anie.202103548.
2021
Variation in the vulnerability of mice expressing human superoxide dismutase 1 to prion-like seeding: a study of the influence of primary amino acid sequence
Acta Neuropathologica Communications. 9(1) [DOI] 10.1186/s40478-021-01191-w. [PMID] 34016165.
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
IL-10 based immunomodulation initiated at birth extends lifespan in a familial mouse model of amyotrophic lateral sclerosis.
Scientific reports. 10(1) [DOI] 10.1038/s41598-020-77564-3. [PMID] 33257786.
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
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.
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
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.
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.
2019
PMP22 Regulates Cholesterol Trafficking and ABCA1-Mediated Cholesterol Efflux
The Journal of Neuroscience. 39(27):5404-5418 [DOI] 10.1523/jneurosci.2942-18.2019.
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.
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
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
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
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
Prion-like Spreading in Tauopathies.
Biological psychiatry. 83(4):337-346 [DOI] 10.1016/j.biopsych.2017.04.003. [PMID] 28506438.
2018
Short Aβ peptides attenuate Aβ42 toxicity in vivo
Journal of Experimental Medicine. 215(1):283-301 [DOI] 10.1084/jem.20170600. [PMID] 29208777.
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
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.
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
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
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.
2016
Generation of a new transgenic mouse model for assessment of tau gene silencing therapies.
Alzheimer's research & therapy. 8(1) [DOI] 10.1186/s13195-016-0202-1. [PMID] 27593210.
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.
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
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
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
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
Vulnerability of newly synthesized proteins to proteostasis stress.
Journal of cell science. 129(9):1892-901 [DOI] 10.1242/jcs.176479. [PMID] 27026526.
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
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
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
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.
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
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
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.
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
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
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.
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
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.
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
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
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
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.
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
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.
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
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
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
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
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
Thinking laterally about neurodegenerative proteinopathies.
The Journal of clinical investigation. 123(5):1847-55 [DOI] 10.1172/JCI66029. [PMID] 23635781.
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.
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
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
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
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.
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
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.
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
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
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.
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
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
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
Structural and biophysical properties of the pathogenic SOD1 variant H46R/H48Q.
Biochemistry. 48(15):3436-47 [DOI] 10.1021/bi8021735. [PMID] 19227972.
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.
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
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
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
Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases.
Neurobiology of aging. 29(4):586-97 [PMID] 17316906.
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.
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
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.
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.
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.
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.
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.
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(Suppl 1):1-269 [PMID] 18085482.
2007
Investigation of RNA interference to suppress expression of full-length and fragment human huntingtin.
Neuromolecular medicine. 9(2):145-55 [PMID] 17627034.
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.
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.
2006
Amyotrophic lateral sclerosis–are microglia killing motor neurons?
The New England journal of medicine. 355(15):1611-3 [PMID] 17035656.
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.
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.
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.
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.
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.
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.
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.
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.
2005
Persistent amyloidosis following suppression of Abeta production in a transgenic model of Alzheimer disease.
PLoS medicine. 2(12) [PMID] 16279840.
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.
2004
APP processing and amyloid deposition in mice haplo-insufficient for presenilin 1.
Neurobiology of aging. 25(7):885-92 [PMID] 15212842.
2004
Environmental, pharmacological, and genetic modulation of the HD phenotype in transgenic mice.
Experimental neurology. 187(1):137-49 [PMID] 15081595.
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.
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.
2003
APP processing and synaptic function.
Neuron. 37(6):925-37 [PMID] 12670422.
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.
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.
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.
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.
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.
2002
Abeta deposition is essential to AD neuropathology.
Journal of Alzheimer's disease : JAD. 4(3):133-8 [PMID] 12226531.
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.
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.
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.
2002
Expression of stabilized beta-catenin in differentiated neurons of transgenic mice does not result in tumor formation.
BMC cancer. 2 [PMID] 12460454.
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.
2002
Genetically engineered mouse models of neurodegenerative diseases.
Nature neuroscience. 5(7):633-9 [PMID] 12085093.
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.
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.
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.
2002
Transgenic mouse models of neurodegenerative disease: opportunities for therapeutic development.
Current neurology and neuroscience reports. 2(5):457-64 [PMID] 12169227.
2001
BACE1 is the major beta-secretase for generation of Abeta peptides by neurons.
Nature neuroscience. 4(3):233-4 [PMID] 11224536.
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.
2001
Co-expression of multiple transgenes in mouse CNS: a comparison of strategies.
Biomolecular engineering. 17(6):157-65 [PMID] 11337275.
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.
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.
2001
Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington’s disease.
Annals of neurology. 50(1):112-7 [PMID] 11456300.
2001
Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA.
Neurobiology of disease. 8(3):405-18 [PMID] 11442350.
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.
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.
2000
Amyloid precursor proteins inhibit heme oxygenase activity and augment neurotoxicity in Alzheimer’s disease.
Neuron. 28(2):461-73 [PMID] 11144356.
2000
Decreased expression of striatal signaling genes in a mouse model of Huntington’s disease.
Human molecular genetics. 9(9):1259-71 [PMID] 10814708.
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.
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.
1999
Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.
Neuron. 24(1):275-86 [PMID] 10677044.
1999
Polyglutamine pathogenesis.
Philosophical transactions of the Royal Society of London. Series B, Biological sciences. 354(1386):1005-11 [PMID] 10434299.
1999
SOD1 rescues cerebral endothelial dysfunction in mice overexpressing amyloid precursor protein.
Nature neuroscience. 2(2):157-61 [PMID] 10195200.
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.
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.
1998
Alzheimer disease–when and why?
Nature genetics. 19(4):314-6 [PMID] 9697686.
1998
Alzheimer’s disease: genetic studies and transgenic models.
Annual review of genetics. 32:461-93 [PMID] 9928488.
1998
An Alzheimer’s disease-linked PS1 variant rescues the developmental abnormalities of PS1-deficient embryos.
Neuron. 20(3):603-9 [PMID] 9539132.
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.
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.
1998
Effects of PS1 deficiency on membrane protein trafficking in neurons.
Neuron. 21(5):1213-21 [PMID] 9856475.
1998
Familial amyotrophic lateral sclerosis and Alzheimer’s disease. Transgenic models.
Advances in experimental medicine and biology. 446:145-59 [PMID] 10079842.
1998
Genetic neurodegenerative diseases: the human illness and transgenic models.
Science (New York, N.Y.). 282(5391):1079-83 [PMID] 9804539.
1998
Inherited neurodegenerative diseases and transgenic models.
Laboratory animal science. 48(6):604-10 [PMID] 10090084.
1998
Metabolism of presenilin 1: influence of presenilin 1 on amyloid precursor protein processing.
Neurobiology of aging. 19(1 Suppl):S15-8 [PMID] 9562461.
1998
Stable association of presenilin derivatives and absence of presenilin interactions with APP.
Neurobiology of disease. 4(6):438-53 [PMID] 9666482.
1998
Transgenic mouse models of Alzheimer’s disease and amyotrophic lateral sclerosis.
Brain pathology (Zurich, Switzerland). 8(4):735-57 [PMID] 9804381.
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.
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.
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.
1997
Amyotrophic lateral sclerosis and Alzheimer disease. Lessons from model systems.
Revue neurologique. 153(8-9):484-95 [PMID] 9683997.
1997
Endoproteolytic processing and stabilization of wild-type and mutant presenilin.
The Journal of biological chemistry. 272(39):24536-41 [PMID] 9305918.
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.
1997
Genetic modification of the phenotypes produced by amyloid precursor protein overexpression in transgenic mice.
Human molecular genetics. 6(11):1951-9 [PMID] 9302276.
1997
Hyperaccumulation of FAD-linked presenilin 1 variants in vivo.
Nature medicine. 3(7):756-60 [PMID] 9212102.
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.
1996
Endoproteolysis of presenilin 1 and accumulation of processed derivatives in vivo.
Neuron. 17(1):181-90 [PMID] 8755489.
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.
1996
Inherited neurodegenerative diseases and transgenic models.
Brain pathology (Zurich, Switzerland). 6(4):467-80 [PMID] 8944317.
1996
Loss of functional prion protein: a role in prion disorders?
Chemistry & biology. 3(8):619-21 [PMID] 8807894.
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.
1996
Motor neuron disease and model systems: aetiologies, mechanisms and therapies.
Ciba Foundation symposium. 196:3-13; discussion 13 [PMID] 8866125.
1996
Neurodegenerative diseases and model systems.
Cold Spring Harbor symposia on quantitative biology. 61:725-38 [PMID] 9246498.
1996
Protein topology of presenilin 1.
Neuron. 17(5):1023-30 [PMID] 8938133.
1996
Transgenic models of neurodegenerative diseases.
Current opinion in neurobiology. 6(5):651-60 [PMID] 8937830.
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.
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.
1995
Cellular and molecular biology of Alzheimer’s disease and animal models.
Neuroimaging clinics of North America. 5(1):59-68 [PMID] 7743085.
1995
Motor neuron disease caused by mutations in superoxide dismutase 1.
Current opinion in neurology. 8(4):294-301 [PMID] 7582045.
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.
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.
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.
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.
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.
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.
1993
Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor.
Glycobiology. 3(4):319-29 [PMID] 7691278.
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.
1992
Evidence for synthesis of scrapie prion proteins in the endocytic pathway.
The Journal of biological chemistry. 267(23):16188-99 [PMID] 1353761.
1992
Synthesis and trafficking of prion proteins in cultured cells.
Molecular biology of the cell. 3(8):851-63 [PMID] 1356522.
1992
Toxicity of synthetic A beta peptides and modeling of Alzheimer’s disease.
Neurobiology of aging. 13(5):623-5 [PMID] 1461354.
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.
1990
Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C.
Biochemistry. 29(22):5405-12 [PMID] 1974460.
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.
1989
Asparagine-linked glycosylation of the scrapie and cellular prion proteins.
Archives of biochemistry and biophysics. 274(1):1-13 [PMID] 2505674.
1989
Inhibition of avian retrovirus protein synthesis in the presence of host cellular mRNA.
Archives of virology. 107(3-4):261-71 [PMID] 2554857.
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.
1988
Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins.
Journal of virology. 62(5):1558-64 [PMID] 3282080.
1987
Scrapie prion protein contains a phosphatidylinositol glycolipid.
Cell. 51(2):229-40 [PMID] 2444340.
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.
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.

Grants

Jul 2023 ACTIVE
Immunotherapy targeting the HPA axis in Alzheimers disease
Role: Co-Investigator
Funding: EMORY UNIV via NATL INST OF HLTH NIA
Sep 2022 ACTIVE
Deciphering tau phosphorylation and Abeta/tau strain interactions in Alzheimers pathogenesis
Role: Principal Investigator
Funding: NATL INST OF HLTH NIA
Apr 2022 ACTIVE
Characterization of new CamK2-tau strains of mice to model dementia
Role: Co-Investigator
Funding: FL DEPT OF HLTH ED ETHEL MOORE ALZHEIMER
Dec 2021 ACTIVE
Modeling the progression of SOD1-linked motor neuron disease
Role: Principal Investigator
Funding: NATL INST OF HLTH NINDS
Sep 2021 ACTIVE
Developing new conditional models to study tauopathy, amyloidosis, and their interaction
Role: Principal Investigator
Funding: NATL INST OF HLTH NINDS
Jan 2020 – Dec 2022
Provenance Initiatives Fund: Towards the identification of new therapies for ALS
Role: Principal Investigator
Funding: UF FOUNDATION
Sep 2019 – Aug 2021
Developing new conditional models to study tauopathy, amyloidosis, and their interaction
Role: Principal Investigator
Funding: NATL INST OF HLTH NINDS
Sep 2018 ACTIVE
Prion and non-prion induction mechanisms of alpha-synuclein pathology
Role: Principal Investigator
Funding: NATL INST OF HLTH NINDS
Jun 2018 – Aug 2023
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: Principal Investigator
Funding: AMYOTROPHIC LATERAL SCLEROSIS ASSOC
Aug 2015 – May 2021
University of Florida – Mt. Sinai Medical Center AD Research Center
Role: Co-Investigator
Funding: NATL INST OF HLTH NIA
Jul 2015 – Dec 2021
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 the progressive spread of ALS symptoms
Role: Principal Investigator
Funding: US ARMY MED RES ACQUISITION
Jun 2015 – May 2017
Role of Misfolded Wild-Type SOD1 in Cases of Sporadic ALS
Role: Principal Investigator
Funding: JOHNS HOPKINS UNIVERSITY
May 2015 – Apr 2021
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 response
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 – Jun 2023
SantaFe HealthCare Alzheimer's Disease Research Center
Role: Principal Investigator
Funding: UF FOUNDATION
Jul 2006 ACTIVE
BRAIN AND SPINAL CORD INJURY RESEARCH
Role: Project Manager
Funding: FL DEPT OF HLTH

Contact Details

Phones:
Business:
(352) 273-9664
Emails:
Business:
drb1@ufl.edu
Addresses:
Business Mailing:
PO Box 100159
GAINESVILLE FL 32610
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