Jonathan D Cooper, Ph.D.  cooperjd@wustl.edu

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Professor of Pediatrics, Genetics and Genomic Medicine
NeurologyGenetics and Genomic MedicinePatient Oriented Research UnitGenetics

phone: (314) 454-6093

Education

  • BSc, Honors, University of Sheffield, UK1986
  • PhD, University of Bristol, UK1990

Training

  • MRC Postdoctoral Training Fellow, University of Cambridge1990 - 1993
  • Postdoctoral Fellow, Max-Planck-Institute for Psychiatry1993 - 1996
  • Senior Postdoctoral Fellow, University of California San Francisco & Stanford University1996 - 2000

Honors and Awards

  • Anatomical Society of Great Britain and Ireland Undergraduate Prize1986 - 1987
  • Medical Research Council Postdoctoral Training Fellowship1990 - 1993
  • Human Capital and Mobility Fellowship of the European Commission1994 - 1996
  • Edwin Boldrey Award of the San Francisco Neurological Society for Excellence in Basic Science Neurological Research1999
  • NCL Stiftung Research Award2012
  • Institute of Psychiatry, Psychology and Neuroscience, Lifetime Achievement Award for excellence in Postgraduate Research Education2016

Recent Publications view all (149)


Publication Co-Authors

  1. Brain transplantation of genetically corrected Sanfilippo type B Neural Stem Cells induces partial cross-correction of the disease (In press) Molecular Therapy - Methods & Clinical Development. 2022. 
  2. Cross-species efficacy of enzyme replacement therapy for CLN1 disease in mice and sheep. J Clin Invest. . 2022;132:e163107. doi:doi: 10.1172/JCI163107  PMID:36040802 
  3. Modelling Neurological Diseases in Large Animals: Criteria for Model Selection and Clinical Assessment. Cells. 2022;11(17):2641. doi:doi: 10.3390/cells11172641  PMID:36078049 
  4. Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures. Sci Rep. 2022;12(1):11286. doi:doi: 10.1038/s41598-022-15134-5  PMID:35789177 
  5. Tralesinidase Alfa Enzyme Replacement Therapy Prevents Disease Manifestations in a Canine Model of Mucopolysaccharidosis Type IIIB. J Pharmacol Exp Ther. 2022;382(3):277-286. doi:doi: 10.1124/jpet.122.001119  PMID:35717448 
  6. Glial pathology and its contribution to the pathogenesis of the neuronal ceroid lipofuscinoses Front Neurol. . 2022;13:886567. doi:doi: 10.3389/fneur.2022.886567  PMCID:PMC9013902  PMID:35444603 
  7. Cortical interneuron loss and seizure generation as novel clinically relevant phenotypes in Cln2R207X mice BioRxiv . 2022. doi:10.1101/2022.03.11.483984  
  8. Neuropathology of murine Sanfilippo D syndrome. Mol Genet Metab. 2021;134(4):323-329. PMID:34844863 
  9. Brain volume in chronic ketamine users - relationship to sub-threshold psychotic symptoms and relevance to schizophrenia. Psychopharmacology (Berl). 2021. PMID:34228135 
  10. Biochemical evaluation of intracerebroventricular rhNAGLU-IGF2 enzyme replacement therapy in neonatal mice with Sanfilippo B syndrome. Mol Genet Metab. 2021;133(2):185-192. PMCID:PMC8195848  PMID:33839004 
  11. Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice. Mol Ther Methods Clin Dev. 2021;20:497-507. PMCID:PMC7887332  PMID:33665223 
  12. Spinal manifestations of CLN1 disease start during the early postnatal period. Neuropathol Appl Neurobiol. 2021;47(2):251-267. PMCID:PMC7867600  PMID:32841420 
  13. Central nervous system pathology in preclinical MPS IIIB dogs reveals progressive changes in clinically relevant brain regions. Sci Rep. 2020;10(1):20365. PMCID:PMC7684310  PMID:33230178 
  14. Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease. Sci Rep. 2020;10(1):15157. PMCID:PMC7495486  PMID:32938982 
  15. Future perspectives: What lies ahead for Neuronal Ceroid Lipofuscinosis research? Biochim Biophys Acta Mol Basis Dis. 2020;1866(9):165681. PMID:31926264 
  16. Pathomechanisms in the neuronal ceroid lipofuscinoses. Biochim Biophys Acta Mol Basis Dis. 2020;1866(9):165570. PMID:31678162 
  17. Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice. Lipids. 2020. doi:10.1002/lipd.12261  PMID:32537944 
  18. Generation of light-producing somatic-transgenic mice using adeno-associated virus vectors. Sci Rep. 2020;10(1):2121. PMCID:PMC7005886  PMID:32034258 
  19. Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3 -/- Mice. Front Neurol. 2019;10:963. PMCID:PMC6749847  PMID:31572287 
  20. CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease). Sci Rep. 2019;9(1):9891. PMCID:PMC6616324  PMID:31289301 
  21. Clinical challenges and future therapeutic approaches for neuronal ceroid lipofuscinosis. Lancet neurology. 2019;18(1):107-116. PMID:30470609 
  22. Genetically Corrected iPSC-Derived Neural Stem Cell Grafts Deliver Enzyme Replacement to Affect CNS Disease in Sanfilippo B Mice. Mol Ther Methods Clin Dev. 2018;10:113-127. PMCID:PMC6076361  PMID:30101150 
  23. Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis. Acta Neuropathol Commun. 2018. PMID:30089511 
  24. Trehalose reduces retinal degeneration, neuroinflammation and storage burden caused by a lysosomal hydrolase deficiency. Autophagy. 2018;1-16. PMID:29916295 
  25. Fetal gene therapy for neurodegenerative disease of infants. Nat Med. 2018. PMID:30013199 
  26. A Humoral Immune Response Alters the Distribution of Enzyme Replacement Therapy in Murine Mucopolysaccharidosis Type I. Mol Ther Methods Clin Dev. 2018;8:42-51. PMCID:PMC5684429  PMID:29159202 
  27. Intravenous administration of scAAV9-Hexb normalizes lifespan and prevents pathology in Sandhoff disease mice. Hum Mol Genet. 2018;27(6):954-968. PMID:29325092 
  28. Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons. Acta Neuropathol Commun. 2017;5(1):74. PMCID:PMC5645909  PMID:29041969 
  29. Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo. Sci Rep. 2017;7(1):12412. PMCID:PMC5622084  PMID:28963550 
  30. Partial rescue of neuropathology in the murine model of PKU following administration of recombinant phenylalanine ammonia lyase (pegvaliase). Mol Genet Metab. 2017;122(1-2):33-35. PMID:28506393 
  31. Progress toward Fulfilling the Potential of Immunomodulation in Childhood Neurodegeneration? Mol Ther. 2017;25(8):1743-1745. PMCID:PMC5542794  PMID:28625570 
  32. Synergistic effects of treating the spinal cord and brain in CLN1 disease. Proc Natl Acad Sci U S A. 2017;114(29):E5920-E5929. PMCID:PMC5530669  PMID:28673981 
  33. Evolution of structural abnormalities in the rat brain following in utero exposure to maternal immune activation: A longitudinal in vivo MRI study. Brain Behav Immun. 2017;63:50-59. PMCID:PMC5441572  PMID:27940258 
  34. Corrigendum: mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases. Nat Commun. 2017;8:15793. PMCID:PMC5474731  PMID:28607479 
  35. Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3). Ann Neurol. 2016;80(6):909-923. PMCID:PMC5215570  PMID:27804148 
  36. Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): Expert recommendations for early detection and laboratory diagnosis. Mol Genet Metab. 2016;119(1-2):160-7. PMID:27553878 
  37. Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease). PLoS One. 2016;11(6):e0157365. PMCID:PMC4915684  PMID:27327661 
  38. Non-invasive molecular imaging of inflammatory macrophages in allograft rejection. EJNMMI Res. 2015;5(1):69. PMCID:PMC4661159  PMID:26611870 
  39. Longitudinal in vivo maturational changes of metabolites in the prefrontal cortex of rats exposed to polyinosinic-polycytidylic acid in utero. Eur Neuropsychopharmacol. 2015;25(12):2210-20. PMID:26475576 
  40. Microglial activation in the rat brain following chronic antipsychotic treatment at clinically relevant doses. Eur Neuropsychopharmacol. 2015;25(11):2098-107. PMID:26321204 
  41. Towards a new understanding of NCL pathogenesis. Biochim Biophys Acta. 2015;1852(10 Pt B):2256-61. PMID:26026924 
  42. Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis. Mol Genet Metab. 2015;116(1-2):98-105. PMID:25982063 
  43. Systemic gene delivery following intravenous administration of AAV9 to fetal and neonatal mice and late-gestation nonhuman primates. FASEB J. 2015;29(9):3876-88. PMCID:PMC4560173  PMID:26062602 
  44. A novel, long-lived, and highly engraftable immunodeficient mouse model of mucopolysaccharidosis type I. Mol Ther Methods Clin Dev. 2015;2:14068. PMCID:PMC4449030  PMID:26052536 
  45. Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis. Mol Genet Metab. 2015;114(2):281-93. PMID:25257657 
  46. Quantifying storage material accumulation in tissue sections. Methods Cell Biol. 2015;126:349-56. PMID:25665454 
  47. Guidelines for incorporating scientific knowledge and practice on rare diseases into higher education: neuronal ceroid lipofuscinoses as a model disorder. Biochimica et biophysica acta. 2015;1852(10 Pt B):2316-23. 
  48. An anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosis. J Neurosci. 2014;34(39):13077-82. PMCID:PMC4172802  PMID:25253854 
  49. Behavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV. Acta Neuropathol Commun. 2014;2:133. PMCID:PMC4173007  PMID:25200117 
  50. Reduced cortical volume and elevated astrocyte density in rats chronically treated with antipsychotic drugs-linking magnetic resonance imaging findings to cellular pathology. Biol Psychiatry. 2014;75(12):982-90. PMID:24143881 
  51. Partial correction of the CNS lysosomal storage defect in a mouse model of juvenile neuronal ceroid lipofuscinosis by neonatal CNS administration of an adeno-associated virus serotype rh.10 vector expressing the human CLN3 gene. Hum Gene Ther. 2014;25(3):223-39. PMCID:PMC3955974  PMID:24372003 
  52. A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease. PLoS One. 2013;8(11):e78694. PMCID:PMC3815212  PMID:24223841 
  53. Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease). Biochim Biophys Acta. 2013;1832(11):1906-9. PMCID:PMC4573397  PMID:23747979 
  54. NCL disease mechanisms. Biochim Biophys Acta. 2013;1832(11):1882-93. PMID:23707513 
  55. Special issue: molecular basis of the NCLs. Biochim Biophys Acta. 2013;1832(11):1793-4. PMID:23727410 
  56. Regional brain atrophy in mouse models of neuronal ceroid lipofuscinosis: a new rostrocaudal perspective. J Child Neurol. 2013;28(9):1117-22. PMID:24014506 
  57. A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation. Brain. 2013;136(Pt 5):1488-507. PMID:23587805 
  58. Cathepsin F mutations cause Type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosis. Hum Mol Genet. 2013;22(7):1417-23. PMCID:PMC3596852  PMID:23297359 
  59. Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis. Brain. 2013;136(Pt 4):1083-101. PMID:23485853 
  60. Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice. Dis Model Mech. 2013;6(2):342-57. PMCID:PMC3597017  PMID:23065637 
  61. Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion. Acta Neuropathol.. 2013;125(2):273-88. PMID:22961620 
  62. Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease. 2012;63(5):769-75. PMID:22683643 
  63. Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice. Neurobiology of Disease. 2012;47(3):444-57. PMID:22569358 
  64. In utero administration of Ad5 and AAV pseudotypes to the fetal brain leads to efficient, widespread and long-term gene expression. Gene Ther. . 2012;19(9):936-46. PMID:22071970 
  65. Galactolipid deficiency in the early pathogenesis of neuronal ceroid lipofuscinosis model Cln8mnd : implications to delayed myelination and oligodendrocyte maturation. Neuropathol Appl Neurobiol. 2012;38(5):471-86. PMID:22044361 
  66. Contrasting effects of haloperidol and lithium on rodent brain structure: a magnetic resonance imaging study with postmortem confirmation. Biol Psychiatry. 2012;71(10):855-63. PMID:22244831 
  67. Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism. Neurobiol Dis. 2012;46(1):19-29. PMID:22182690 
  68. Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice. Neurobiol Dis. 2012;45(3):1086-100. PMCID:PMC3657200  PMID:22198570 
  69. Synergistic effects of central nervous system-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis. Annals of neurology. 2012;71(6):797-804. 
  70. Early microglial activation precedes neuronal loss in the brain of the Cstb-/- mouse model of progressive myoclonus epilepsy, EPM1 Journal of neuropathology and experimental neurology. 2012;71(1):40-53. 
  71. Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis. NIHMSID: NIHMS379947 Molecular genetics and metabolism. 2012;107(1-2):213-21. 
  72. Combination small molecule PPT1 mimetic and CNS-directed gene therapy as a treatment for infantile neuronal ceroid lipofuscinosis. NIHMSID: NIHMS609910 Journal of inherited metabolic disease. 2012;35(5):847-57. 
  73. Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIB PloS one. 2012;7(4):e35787. 
  74. Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis. PLoS One. 2012;7(4):e35493. PMCID:PMC3335005  PMID:22536393 
  75. Spatial and temporal correlation between neuron loss and neuroinflammation in a mouse model of neuronopathic Gaucher disease Human molecular genetics. 2011;20(7):1375-86. 
  76. A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses Journal of biomolecular NMR. 2011;49(3-4):175-84. 
  77. Insulin receptor substrate 2 is a negative regulator of memory formation Learning & memory (Cold Spring Harbor, N.Y.). 2011;18(6):375-83. 
  78. Loss of amyloid precursor protein in a mouse model of Niemann-Pick type C disease exacerbates its phenotype and disrupts tau homeostasis. . 2011; 42(3):349-59. Neurobiology of disease. 2011;42(3):349-59. 
  79. Immunosuppression alters disease severity in juvenile Batten disease mice Journal of neuroimmunology. 2011;230(1-2):169-72. 
  80. Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten disease NIHMSID: NIHMS252642 Neuropharmacology. 2011;60(2-3):405-9. 
  81. Morphologic and functional correlates of synaptic pathology in the cathepsin D knockout mouse model of congenital neuronal ceroid lipofuscinosis NIHMSID: UKMS36995 Journal of neuropathology and experimental neurology. 2011;70(12):1089-96. 
  82. Intravenous administration of AAV2/9 to the fetal and neonatal mouse leads to differential targeting of CNS cell types and extensive transduction of the nervous system FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 2011;25(10):3505-18. 
  83. The neuronal ceroid lipofuscinoses: the same, but different? Biochemical Society transactions. 2010;38(6):1448-52. PMID:21118105 
  84. In utero gene transfer to the mouse nervous system. Biochemical Society transactions. 2010;38(6):1489-93. PMID:21118113 
  85. DHHC5 interacts with PDZ domain 3 of post-synaptic density-95 (PSD-95) protein and plays a role in learning and memory. . 2010; 285(17):13022-31. The Journal of biological chemistry. 2010;285(17):13022-31. 
  86. Current therapies for the soluble lysosomal forms of neuronal ceroid lipofuscinosis. Biochemical Society transactions. 2010;38(6):1484-8. 
  87. The RGD-containing peptide fragment of osteopontin protects tyrosine hydroxylase positive cells against toxic insult in primary ventral mesencephalic cultures and in the rat substantia nigra. . 2010; 114(6):1792-804. Journal of neurochemistry. 2010;114(6):1792-804. 
  88. Anti-inflammatory impact of minocycline in a mouse model of tauopathy Frontiers in psychiatry. 2010;1:136. 
  89. Effects of cis-regulatory variation differ across regions of the adult human brain. . 2010; 19(22):4490-6. Human molecular genetics. 2010;19(22):4490-6. 
  90. Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB. . 2010; 209(2):212-20. Behavioural brain research. 2010;209(2):212-20. 
  91. Distinct neuropathologic phenotypes after disrupting the chloride transport proteins ClC-6 or ClC-7/Ostm1 Journal of neuropathology and experimental neurology. 2010;69(12):1228-46. 
  92. Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL. Neurobiol Dis. 2009;34(2):308-19. PMCID:PMC2704904  PMID:19385065 
  93. Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis NIHMSID: NIHMS97005 Brain research. 2009;1266:93-107. 
  94. An immunohistochemical and stereological analysis of PSI-induced nigral neuronal degeneration in the rat. Journal of neurochemistry 2009;109(1):52-9. 
  95. Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease Human molecular genetics. 2009;18(21):4066-80. 
  96. Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse NIHMSID: NIHMS95940 Experimental neurology. 2009;217(1):124-35. 
  97. Efficient gene delivery to the adult and fetal CNS using pseudotyped non-integrating lentiviral vectors Gene therapy. 2009;16(4):509-20. 
  98. Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis Cell stem cell. 2009;5(3):310-9. 
  99. Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis. Neurobiol Dis. 2008;32(1):50-65. PMCID:PMC2647510  PMID:18634879 
  100. Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases. BMC Genomics. 2008;9:146. PMCID:PMC2323392  PMID:18371231 
  101. Immune system irregularities in lysosomal storage disorders. Acta neuropathologica. 2008;115(2):159-74. 
  102. Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis Molecular therapy : the journal of the American Society of Gene Therapy. 2008;16(4):649-56. 
  103. Synaptic changes in the thalamocortical system of cathepsin D-deficient mice: a model of human congenital neuronal ceroid-lipofuscinosis. . 2008; 67(1):16-29 Journal of neuropathology and experimental neurology. 2008;67(1):16-29. 
  104. Moving towards therapies for juvenile Batten disease? Experimental neurology. 2008;211(2):329-31. 
  105. Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis Neurobiology of disease. 2007;25(1):150-62. 
  106. IgG entry and deposition are components of the neuroimmune response in Batten disease Neurobiology of disease. 2007;25(2):239-51. 
  107. Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosis Brain research. 2007;1162:98-112. 
  108. Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis. Biochimica et biophysica acta. 2006;1762(10):873-89. 
  109. CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis Molecular therapy : the journal of the American Society of Gene Therapy. 2006;13(3):538-47. 
  110. Distinct patterns of serum immunoreactivity as evidence for multiple brain-directed autoantibodies in juvenile neuronal ceroid lipofuscinosis Neuropathology and applied neurobiology. 2006;32(5):469-82. 
  111. Activation of non-neuronal cells within the prenatal developing brain of sheep with neuronal ceroid lipofuscinosis Brain pathology (Zurich, Switzerland). 2006;16(2):110-6. 
  112. Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons Neurobiology of disease. 2006;22(2):284-93. 
  113. Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7/8 knock-in mouse model of Batten disease. Neurobiol Dis. 2005;20(3):823-36. PMID:16006136 
  114. Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6). Neurobiol Dis. 2005;20(1):49-63. PMID:16137566 
  115. High resolution 1H NMR-based metabolomics indicates a neurotransmitter cycling deficit in cerebral tissue from a mouse model of Batten disease The Journal of biological chemistry. 2005;280(52):42508-14. 
  116. FGFR3 regulates brain size by controlling progenitor cell proliferation and apoptosis during embryonic development Developmental biology. 2005;279(1):73-85. 
  117. Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation. Brain Res. 2004;1023(2):231-42. PMID:15374749 
  118. Hippocampal pathology in the human neuronal ceroid-lipofuscinoses: distinct patterns of storage deposition, neurodegeneration and glial activation. Brain Pathol. 2004;14(4):349-57. PMCID:PMC8095893  PMID:15605981 
  119. Selectivity and types of cell death in the neuronal ceroid lipofuscinoses. Brain pathology (Zurich, Switzerland). 2004;14(1):86-96. 
  120. Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis Neurobiology of disease. 2004;16(2):346-59. 
  121. Adeno-associated virus 2-mediated gene therapy decreases autofluorescent storage material and increases brain mass in a murine model of infantile neuronal ceroid lipofuscinosis Neurobiology of disease. 2004;16(2):360-69. 
  122. p38alpha stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis Molecular and cellular neurosciences. 2004;26(2):354-64. 
  123. A novel somatodendritic marker defined by a peptide derived from the ALS2 protein Neuroreport. 2004;15(14):2155-9. 
  124. Trafficking the NGF signal: implications for normal and degenerating neurons Progress in brain research. 2004;146:3-23. 
  125. Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues. Biochem J. 2003;376(Pt 2):369-76. PMCID:PMC1223781  PMID:12946273 
  126. The neuronal adaptor protein X11alpha reduces Abeta levels in the brains of Alzheimer's APPswe Tg2576 transgenic mice The Journal of biological chemistry. 2003;278(47):47025-9. 
  127. Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis. Current opinion in neurology. 2003;16(2):121-8. 
  128. Identification of a novel, membrane-associated neuronal kinase, cyclin-dependent kinase 5/p35-regulated kinase The Journal of neuroscience : the official journal of the Society for Neuroscience. 2003;23(12):4975-83. 
  129. Expression of the Fe65 adapter protein in adult and developing mouse brain. Neuroscience. 2002;115(3):951-60. 
  130. An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease Human molecular genetics. 2002;11(12):1421-31. 
  131. Failed retrograde transport of NGF in a mouse model of Down's syndrome: reversal of cholinergic neurodegenerative phenotypes following NGF infusion Proceedings of the National Academy of Sciences of the United States of America. 2001;98(18):10439-44. 
  132. Neurotrophic factors as potential therapeutic agents in neuronal ceroid lipofuscinosis. Advances in genetics. . 2001;45:169-82. 
  133. Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected]. Neurobiol Dis. 1999;6(5):321-34. PMID:10527801 
  134. Apparent loss and hypertrophy of interneurons in a mouse model of neuronal ceroid lipofuscinosis: evidence for partial response to insulin-like growth factor-1 treatment The Journal of neuroscience : the official journal of the Society for Neuroscience. 1999;19(7):2556-67. 
  135. Ganglioside GM1 potentiates NGF action on axotomised medial septal cholinergic neurons Brain research. 1998;812(1-2):76-80. 
  136. Absence of p75NTR causes increased basal forebrain cholinergic neuron size, choline acetyltransferase activity, and target innervation The Journal of neuroscience : the official journal of the Society for Neuroscience. 1997;17(20):7594-605. 
  137. Increased vulnerability of septal cholinergic neurons to partial loss of target neurons in aged rats Neuroscience. 1996;75(1):29-35. 
  138. Delayed death of septal cholinergic neurons after excitotoxic ablation of hippocampal neurons during early postnatal development in the rat Experimental neurology. 1996;139(1):143-55. 
  139. Reduced retrograde labelling with fluorescent tracer accompanies neuronal atrophy of basal forebrain cholinergic neurons in aged rats Neuroscience. 1996;75(1):19-27. 
  140. Inactivation of bcl-2 results in progressive degeneration of motoneurons, sympathetic and sensory neurons during early postnatal development Neuron. 1996;17(1):75-89. 
  141. NMDA potentiates NGF-induced sprouting of septal cholinergic fibres Neuroreport. 1994;5(4):413-6. 
  142. Reduced transport of 125I-nerve growth factor by cholinergic neurons and down-regulated TrkA expression in the medial septum of aged rats Neuroscience. 1994;62(3):625-9. 
  143. Central neuroanatomical organisation of the rat visuomotor system. Prog Neurobiol. 1993;41(2):209-79. PMID:8332752 
  144. Atrophy but not death of adult septal cholinergic neurons after ablation of target capacity to produce mRNAs for NGF, BDNF, and NT3 The Journal of neuroscience; the official journal of the Society for Neuroscience. 1993;13(12):5263-76. 
  145. Trophic factor effects on septal cholinergic neurons. Annals of the New York Academy of Sciences. 1991;640:91-4. 
  146. Projections to the rostral reticular thalamic nucleus in the rat Experimental brain research. 1990;80(1):157-71. 
  147. Afferent and efferent connections of the laterodorsal tegmental nucleus in the rat. Brain research bulletin 1990;25(2):271-84. 
  148. Accurate counting of neurons in frozen sections: some necessary precautions. Journal of anatomy Journal of anatomy. 1988;80(1):13-21. 
  149. A temperature controlled chamber to allow observation and measurement of uptake of fluorochromes into live cells Journal of microscopy. 1987;14(3):329-35. 
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