Nelvagal HR, Hurtado ML, Eaton SL, Kline RA, Lamont DJ, Sands MS, Wishart TM, Cooper JD. Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease. Sci Rep.. 2020;10(1):15157. doi:10.1038/s41598-020-72075-7 PMID:32938982 Nelvagal HR, Dearborn JT, Ostergaard JR, Sands MS, Cooper JD. Spinal manifestations of CLN1 disease start during the early postnatal period. Neuropathol Appl Neurobiol.. 2020. doi:doi: 10.1111/nan.12658 PMID:32841420 Le SQ, Nestrasil I, Kan SH, Egeland M, Cooper JD, Elashoff D, Guo R, Tolar J, Yee JK, Dickson PI. Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice. Lipids. 2020. doi:10.1002/lipd.12261 PMID:32537944 Cooper JD, Mole SE. Future perspectives: What lies ahead for Neuronal Ceroid Lipofuscinosis research? Biochim Biophys Acta Mol Basis Dis. 2020;(1866 (9)):165681. doi:10.1016/j.bbadis.2020.165681. PMID:31926264 Nelvagal HR, Lange J, Takahashi K, Tarczyluk-Wells MA, Cooper JD.. Pathomechanisms in the neuronal ceroid lipofuscinoses. Biochim Biophys Acta Mol Basis Dis.. 2019;(1866(9)):165570. doi:10.1016/j.bbadis.2019.165570 PMID:31678162 Tarczyluk-Wells MA, Salzlechner C, Najafi AR, Lim MJ, Smith D, Platt FM, Williams BP, Cooper JD. Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3 -/- Mice. Frontiers in Neurology. 2019;10:963. doi:10.3389/fneur.2019.00963 PMID:31572287 Eaton SL, Proudfoot C, Lillico SG, Skehel P, Kline RA, Hamer K, Rzechorzek NM, Clutton E, Gregson R, King T, O'Neill CA, Cooper JD, Thompson G, Whitelaw CB, Wishart TM. CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease). Scientific Reports. 2019. PMID:31289301 Mole SE, Anderson G, Band HA, Berkovic SF, Cooper JD, Kleine Holthaus SM, McKay TR, Medina DL, Rahim AA, Schulz A, Smith AJ.. Clinical challenges and future therapeutic approaches for neuronal ceroid lipofuscinosis. Lancet neurology. 2019;18(1):107-116. PMID:30470609 Clarke D, Pearse Y, Kan S, Le SQ, Sanghez V, Cooper JD, Dickson PI, Iacovino M. Genetically Corrected iPSC-Derived Neural Stem Cell Grafts Deliver Enzyme Replacement to Affect CNS Disease in Sanfilippo B Mice. Molecular Therapy: Methods & Clinical Development. 2018;10.
Lange J, Haslett LJ, Lloyd-Evans E, Pocock JM, Sands MS, Williams BP, Cooper JD . Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis. Acta Neuropathol Commun. 2018. PMID:30089511 Lotfi P, Tse DY, Di Ronza A, Seymour ML, Martano G, Cooper JD, Pereira FA, Passafaro M, Wu SM, Sardiello M. Trehalose reduces retinal degeneration, neuroinflammation and storage burden caused by a lysosomal hydrolase deficiency. Autophagy. 2018;1-16. PMID:29916295 Massaro G, Mattar CNZ, Wong AMS, Sirka E, Buckley SMK, Herbert BR, Karlsson S, Perocheau DP, Burke D, Heales S, Richard-Londt A, Brandner S, Huebecker M, Priestman DA, Platt FM, Mills K, Biswas A, Cooper JD, Chan JKY, Cheng SH, Waddington SN, Rahim AA. Fetal gene therapy for neurodegenerative disease of infants. Nat Med. 2018. PMID:30013199 Le SQ, Kan SH, Clarke D, Sanghez V, Egeland M, Vondrak KN, Doherty TM, Vera MU, Iacovino M, Cooper JD, Sands MS, Dickson PI. 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 Niemir N, Rouvière L, Besse A, Vanier MT, Dmytrus J, Marais T, Astord S, Puech JP, Panasyuk G, Cooper JD, Barkats M, Caillaud C. Intravenous administration of scAAV9-Hexb normalizes lifespan and prevents pathology in Sandhoff disease mice. Hum Mol Genet. 2018;27(6):954-968. PMID:29325092 Parviainen L, Dihanich S, Anderson GW, Wong AM, Brooks HR, Abeti R, Rezaie P, Lalli G, Pope S, Heales SJ, Mitchison HM, Williams BP, Cooper JD. 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 Llavero Hurtado M, Fuller HR, Wong AMS, Eaton SL, Gillingwater TH, Pennetta G, Cooper JD, Wishart TM. 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 Goldfinger M, Zeile WL, Corado CR, O'Neill CA, Tsuruda LS, Laipis PJ, Cooper JD. 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 Cooper JD, Nelvagal HR. Progress toward Fulfilling the Potential of Immunomodulation in Childhood Neurodegeneration? Mol Ther. 2017;25(8):1743-1745. PMCID:PMC5542794 PMID:28625570 Shyng C, Nelvagal HR, Dearborn JT, Tyynelä J, Schmidt RE, Sands MS, Cooper JD. 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 Crum WR, Sawiak SJ, Chege W, Cooper JD, Williams SCR, Vernon AC. 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 Palmieri M, Pal R, Nelvagal HR, Lotfi P, Stinnett GR, Seymour ML, Chaudhury A, Bajaj L, Bondar VV, Bremner L, Saleem U, Tse DY, Sanagasetti D, Wu SM, Neilson JR, Pereira FA, Pautler RG, Rodney GG, Cooper JD, Sardiello M. Corrigendum: mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases. Nat Commun. 2017;8:15793. PMCID:PMC5474731 PMID:28607479 Palmieri M, Pal R, Nelvagal HR, Lotfi P, Stinnett GR, Seymour ML, Chaudhury A, Bajaj L, Bondar VV, Bremner L, Saleem U, Tse DY, Sanagasetti D, Wu SM, Neilson JR, Pereira FA, Pautler RG, Rodney GG, Cooper JD, Sardiello M. mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases. Nat Commun. 2017;8:14338. PMCID:PMC5303831 PMID:28165011 Aldrich A, Bosch ME, Fallet R, Odvody J, Burkovetskaya M, Rama Rao KV, Cooper JD, Drack AV, Kielian T. Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3). Ann Neurol. 2016;80(6):909-923. PMCID:PMC5215570 PMID:27804148 Fietz M, AlSayed M, Burke D, Cohen-Pfeffer J, Cooper JD, Dvořáková L, Giugliani R, Izzo E, Jahnová H, Lukacs Z, Mole SE, Noher de Halac I, Pearce DA, Poupetova H, Schulz A, Specchio N, Xin W, Miller N. 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 Wager K, Zdebik AA, Fu S, Cooper JD, Harvey RJ, Russell C. Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease). PLoS One. 2016;11(6):e0157365. PMCID:PMC4915684 PMID:27327661 O'Neill AS, Terry SY, Brown K, Meader L, Wong AM, Cooper JD, Crocker PR, Wong W, Mullen GE. Non-invasive molecular imaging of inflammatory macrophages in allograft rejection. EJNMMI Res. 2015;5(1):69. PMCID:PMC4661159 PMID:26611870 Vernon AC, So PW, Lythgoe DJ, Chege W, Cooper JD, Williams SC, Kapur S. 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 Cotel MC, Lenartowicz EM, Natesan S, Modo MM, Cooper JD, Williams SC, Kapur S, Vernon AC. Microglial activation in the rat brain following chronic antipsychotic treatment at clinically relevant doses. Eur Neuropsychopharmacol. 2015;25(11):2098-107. PMID:26321204 Cooper JD, Tarczyluk MA, Nelvagal HR. Towards a new understanding of NCL pathogenesis. Biochim Biophys Acta. 2015;1852(10 Pt B):2256-61. PMID:26026924 Lu JY, Nelvagal HR, Wang L, Birnbaum SG, Cooper JD, Hofmann SL. 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 Mattar CN, Wong AM, Hoefer K, Alonso-Ferrero ME, Buckley SM, Howe SJ, Cooper JD, Waddington SN, Chan JK, Rahim AA. 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 Mendez DC, Stover AE, Rangel AD, Brick DJ, Nethercott HE, Torres MA, Khalid O, Wong AM, Cooper JD, Jester JV, Monuki ES, McGuire C, Le SQ, Kan SH, Dickson PI, Schwartz PH. 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 Vuillemenot BR, Kennedy D, Cooper JD, Wong AM, Sri S, Doeleman T, Katz ML, Coates JR, Johnson GC, Reed RP, Adams EL, Butt MT, Musson DG, Henshaw J, Keve S, Cahayag R, Tsuruda LS, O'Neill CA. Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis. Mol Genet Metab. 2015;114(2):281-93. PMID:25257657 Cooper JD, Brooks HR, Nelvagal HR. Quantifying storage material accumulation in tissue sections. Methods Cell Biol. 2015;126:349-56. PMID:25665454 Cismondi IA, Kohan R, Adams H, Bond M, Brown R, Cooper JD, de Hidalgo PK, Holthaus SM, Mole SE, Mugnaini J, de Ramirez AM, Pesaola F, Rautenberg G, Platt FM, Noher de Halac I. 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.
Macauley SL, Wong AM, Shyng C, Augner DP, Dearborn JT, Pearse Y, Roberts MS, Fowler SC, Cooper JD, Watterson DM, Sands MS. 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 Grishchuk Y, Sri S, Rudinskiy N, Ma W, Stember KG, Cottle MW, Sapp E, Difiglia M, Muzikansky A, Betensky RA, Wong AM, Bacskai BJ, Hyman BT, Kelleher RJ 3rd, Cooper JD, Slaugenhaupt SA. 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 Vernon AC, Crum WR, Lerch JP, Chege W, Natesan S, Modo M, Cooper JD, Williams SC, Kapur S. 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 Sondhi D, Scott EC, Chen A, Hackett NR, Wong AM, Kubiak A, Nelvagal HR, Pearse Y, Cotman SL, Cooper JD, Crystal RG. 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 Morgan JP, Magee H, Wong A, Nelson T, Koch B, Cooper JD, Weimer JM. 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 Hawkins-Salsbury JA, Cooper JD, Sands MS. Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease). Biochim Biophys Acta. 2013;1832(11):1906-9. PMCID:PMC4573397 PMID:23747979 Palmer DN, Barry LA, Tyynelä J, Cooper JD. NCL disease mechanisms. Biochim Biophys Acta. 2013;1832(11):1882-93. PMID:23707513 Mole SE, Williams RE, Cooper JD. Special issue: molecular basis of the NCLs. Biochim Biophys Acta. 2013;1832(11):1793-4. PMID:23727410 Kühl TG, Dihanich S, Wong AM, Cooper JD. Regional brain atrophy in mouse models of neuronal ceroid lipofuscinosis: a new rostrocaudal perspective. J Child Neurol. 2013;28(9):1117-22. PMID:24014506 Mahmood F, Fu S, Cooke J, Wilson SW, Cooper JD, Russell C. 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 Smith KR, Dahl HH, Canafoglia L, Andermann E, Damiano J, Morbin M, Bruni AC, Giaccone G, Cossette P, Saftig P, Grötzinger J, Schwake M, Andermann F, Staropoli JF, Sims KB, Mole SE, Franceschetti S, Alexander NA, Cooper JD, Chapman HA, Carpenter S, Berkovic SF, Bahlo M. 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 Groh J, Kühl TG, Ip CW, Nelvagal HR, Sri S, Duckett S, Mirza M, Langmann T, Cooper JD, Martini R. 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 Blom T, Schmiedt ML, Wong AM, Kyttälä A, Soronen J, Jauhiainen M, Tyynelä J, Cooper JD, Jalanko A. Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice. Dis Model Mech. 2013;6(2):342-57. PMCID:PMC3597017 PMID:23065637 Mitchell JC, McGoldrick P, Vance C, Hortobagyi T, Sreedharan J, Rogelj B, Tudor EL, Smith BN, Klasen C, Miller CC, Cooper JD, Greensmith L, Shaw CE.. 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 Kovács AD, Saje A, Wong A, Ramji S, Cooper JD, Pearce DA.. Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease. 2012;63(5):769-75. PMID:22683643 Kuronen M, Lehesjoki AE, Jalanko A, Cooper JD, Kopra O.. 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 Rahim AA, Wong AM, Ahmadi S, Hoefer K, Buckley SM, Hughes DA, Nathwani AN, Baker AH, McVey JH, Cooper JD, Waddington SN.. 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 Vernon AC, Natesan S, Crum WR, Cooper JD, Modo M, Williams SC, Kapur S.. 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 Macauley SL, Roberts MS, Wong AM, McSloy F, Reddy AS, Cooper JD, Sands MS. 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.
Tegelberg S, Kopra O, Joensuu T, Cooper JD, Lehesjoki AE. 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.
Hu J, Lu JY, Wong AM, Hynan LS, Birnbaum SG, Yilmaz DS, Streit BM, Lenartowicz EM, Thompson TC, Cooper JD, Hofmann SL. 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.
Thelen M, Damme M, Schweizer M, Hagel C, Wong AM, Cooper JD, Braulke T, Galliciotti G. 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.
Pressey SN, Smith DA, Wong AM, Platt FM, Cooper JD. Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice. Neurobiology of disease. 2012;45(3):1086-100.
Roberts MS, Macauley SL, Wong AM, Yilmas D, Hohm S, Cooper JD, Sands MS. 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.
Schmiedt ML, Blom T, Blom T, Kopra O, Wong A, von Schantz-Fant C, Ikonen E, Kuronen M, Jauhiainen M, Cooper JD, Jalanko A.. Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism. Neurobiology of disease. 2012;46(1):19-29.
Wilkinson FL, Holley RJ, Langford-Smith KJ, Badrinath S, Liao A, Langford-Smith A, Cooper JD, Jones SA, Wraith JE, Wynn RF, Merry CL, Bigger BW. Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIB PloS one. 2012;7(4):e35787.
Kuronen M, Hermansson M, Manninen O, Zech I, Talvitie M, Laitinen T, Gröhn O, Somerharju P, Eckhardt M, Cooper JD, Lehesjoki AE, Lahtinen U, Kopra O. Galactolipid deficiency in the early pathogenesis of neuronal ceroid lipofuscinosis model Cln8mnd : implications to delayed myelination and oligodendrocyte maturation. Neuropathology and applied neurobiology. 2012;38(5):471-86.
Farfel-Becker T, Vitner EB, Pressey SN, Eilam R, Cooper JD, Futerman AH. 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.
Salek RM, Pears MR, Cooper JD, Mitchison HM, Pearce DA, Mortishire-Smith RJ, Griffin JL. A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses Journal of biomolecular NMR. 2011;49(3-4):175-84.
Irvine EE, Drinkwater L, Radwanska K, Al-Qassab H, Smith MA, O'Brien M, Kielar C, Choudhury AI, Krauss S, Cooper JD, Withers DJ, Giese KP. Insulin receptor substrate 2 is a negative regulator of memory formation Learning & memory (Cold Spring Harbor, N.Y.). 2011;18(6):375-83.
Nunes A, Pressey SN, Cooper JD, Soriano S. 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.
Seehafer SS, Ramirez-Montealegre D, Wong AM, Chan CH, Castaneda J, Horak M, Ahmadi SM, Lim MJ, Cooper JD, Pearce DA. Immunosuppression alters disease severity in juvenile Batten disease mice Journal of neuroimmunology. 2011;230(1-2):169-72.
Kovács AD, Saje A, Wong A, Szénási G, Kiricsi P, Szabó E, Cooper JD, Pearce DA. 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.
Koch S, Molchanova SM, Wright AK, Edwards A, Cooper JD, Taira T, Gillingwater TH, Tyynelä J. 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.
Rahim AA, Wong AM, Hoefer K, Buckley SM, Mattar CN, Cheng SH, Chan JK, Cooper JD, Waddington SN. 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.
Cooper JD. The neuronal ceroid lipofuscinoses: the same, but different? Biochemical Society transactions. 2010;38(6):1448-52. PMID:21118105 Rahim AA, Wong AM, Buckley SM, Chan JK, David AL, Cooper JD, Coutelle C, Peebles DM, Waddington SN. In utero gene transfer to the mouse nervous system. Biochemical Society transactions. 2010;38(6):1489-93. PMID:21118113 Li Y, Hu J, Höfer K, Wong AM, Cooper JD, Birnbaum SG, Hammer RE, Hofmann SL. 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.
Wong AM, Rahim AA, Waddington SN, Cooper JD. Current therapies for the soluble lysosomal forms of neuronal ceroid lipofuscinosis. Biochemical Society transactions. 2010;38(6):1484-8.
Iczkiewicz J, Broom L, Cooper JD, Wong AM, Rose S, Jenner. 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.
Garwood CJ, Cooper JD, Hanger DP, Noble W. Anti-inflammatory impact of minocycline in a mouse model of tauopathy Frontiers in psychiatry. 2010;1:136.
Buonocore F, Hill MJ, Campbell CD, Oladimeji PB, Jeffries AR, Troakes C, Hortobagyi T, Williams BP, Cooper JD, Bray NJ. 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.
Canal MM, Wilkinson FL, Cooper JD, Wraith JE, Wynn R, Bigger BW. 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.
Pressey SN, O'Donnell KJ, Stauber T, Fuhrmann JC, Tyynelä J, Jentsch TJ, Cooper JD. 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.
Weimer JM, Benedict JW, Getty AL, Pontikis CC, Lim MJ, Cooper JD, Pearce DA. Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis NIHMSID: NIHMS97005 Brain research. 2009;1266:93-107.
Bukhatwa S, Iravani MM, Zeng BY, Cooper JD, Rose S, Rose S, Jenner P. An immunohistochemical and stereological analysis of PSI-induced nigral neuronal degeneration in the rat. Journal of neurochemistry 2009;109(1):52-9.
von Schantz C, Kielar C, Hansen SN, Pontikis CC, Alexander NA, Kopra O, Jalanko A, Cooper JD. Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL NIHMSID: NIHMS99856 Neurobiology of disease. 2009;34(2):308-19.
Kielar C, Wishart TM, Palmer A, Dihanich S, Wong AM, Macauley SL, Chan CH, Sands MS, Pearce DA, Cooper JD, Gillingwater TH. Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease Human molecular genetics. 2009;18(21):4066-80.
Macauley SL, Wozniak DF, Kielar C, Tan Y, Cooper JD, Sands MS. Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse NIHMSID: NIHMS95940 Experimental neurology. 2009;217(1):124-35.
Rahim AA, Wong AM, Howe SJ, Buckley SM, Acosta-Saltos AD, Elston KE, Ward NJ, Philpott NJ, Cooper JD, Anderson PN, Waddington SN, Thrasher AJ, Raivich G. Efficient gene delivery to the adult and fetal CNS using pseudotyped non-integrating lentiviral vectors Gene therapy. 2009;16(4):509-20.
Tamaki SJ, Jacobs Y, Dohse M, Capela A, Cooper JD, Reitsma M, He D, Tushinski R, Belichenko PV, Salehi A, Mobley W, Gage FH, Huhn S, Tsukamoto AS, Weissman IL, Uchida N. 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.
von Schantz C, Saharinen J, Kopra O, Cooper JD, Gentile M, Hovatta I, Peltonen L, Jalanko A. 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.
Castaneda JA, Lim MJ, Cooper JD, Pearce DA. Immune system irregularities in lysosomal storage disorders. Acta neuropathologica. 2008;115(2):159-74.
Chang M, Cooper JD, Sleat DE, Cheng SH, Dodge JC, Passini MA, Lobel P, Davidson BL. 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.
Oswald MJ, Palmer DN, Kay GW, Barwell KJ, Cooper JD. Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis NIHMSID: NIHMS71086 Neurobiology of disease. 2008;32(1):50-65.
Partanen S, Haapanen A, Kielar C, Pontikis C, Alexander N, Inkinen T, Saftig P, Gillingwater TH, Cooper JD, Tyynelä J. 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.
Cooper JD. Moving towards therapies for juvenile Batten disease? Experimental neurology. 2008;211(2):329-31.
Kielar C, Maddox L, Bible E, Pontikis CC, Macauley SL, Griffey MA, Wong M, Sands MS, Cooper JD. 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.
Lim MJ, Alexander N, Benedict JW, Chattopadhyay S, Shemilt SJ, Guérin CJ, Cooper JD, Pearce DA. IgG entry and deposition are components of the neuroimmune response in Batten disease Neurobiology of disease. 2007;25(2):239-51.
Weimer JM, Benedict JW, Elshatory YM, Short DW, Ramirez-Montealegre D, Ryan DA, Alexander NA, Federoff HJ, Cooper JD, Pearce DA. 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.
Cooper JD, Russell C, Mitchison HM. Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis. Biochimica et biophysica acta. 2006;1762(10):873-89.
Griffey MA, Wozniak D, Wong M, Bible E, Johnson K, Rothman SM, Wentz AE, Cooper JD, Sands MS. 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.
Lim MJ, Beake J, Bible E, Curran TM, Ramirez-Montealegre D, Pearce DA, Cooper JD. 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.
Kay GW, Palmer DN, Rezaie P, Cooper JD. 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.
Weimer JM, Custer AW, Benedict JW, Alexander NA, Kingsley E, Federoff HJ, Cooper JD, Pearce DA. 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.
Oswald MJ, Palmer DN, Kay GW, Shemilt SJ, Rezaie P, Cooper JD. Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6) Neurobiology of disease. 2005;20(1):49-63.
Pontikis CC, Cotman SL, MacDonald ME, Cooper JD. Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7/8 knock-in mouse model of Batten disease Neurobiology of disease. 2005;20(3):823-36.
Pears MR, Cooper JD, Mitchison HM, Mortishire-Smith RJ, Pearce DA, Griffin JL. 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.
Inglis-Broadgate SL, Thomson RE, Pellicano F, Tartaglia MA, Pontikis CC, Cooper JD, Iwata T. FGFR3 regulates brain size by controlling progenitor cell proliferation and apoptosis during embryonic development Developmental biology. 2005;279(1):73-85.
Pontikis CC, Cella CV, Parihar N, Lim MJ, Chakrabarti S, Mitchison HM, Mobley WC, Rezaie P, Pearce DA, Cooper JD. Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation Brain research. 2004;1023(2):231-42.
Mitchison HM, Lim MJ, Cooper JD. Selectivity and types of cell death in the neuronal ceroid lipofuscinoses. Brain pathology (Zurich, Switzerland). 2004;14(1):86-96.
Bible E, Gupta P, Hofmann SL, Cooper JD. 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.
Griffey MA, Bible E, Vogler C, Levy B, Gupta P, Cooper JD, Sands MS. 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.
Ackerley S, Grierson AJ, Banner S, Perkinton MS, Brownlees J, Byers HL, Ward M, Thornhill P, Hussain K, Waby JS, Anderton BH, Cooper JD, Dingwall C, Leigh PN, Shaw CE, Miller CC. 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.
Tyynelä J, Cooper JD, Khan MN, Shemilt SJ, Haltia M. Hippocampal pathology in the human neuronal ceroid-lipofuscinoses: distinct patterns of storage deposition, neurodegeneration and glial activation Brain pathology (Zurich, Switzerland). 2004;14(4):349-57.
Bros V, Shemilt SJ, Cooper JD, Skaper SD, Leigh PN, Gallo JM. A novel somatodendritic marker defined by a peptide derived from the ALS2 protein Neuroreport. 2004;15(14):2155-9.
Delcroix JD, Valletta J, Wu C, Howe CL, Lai CF, Lai CF, Cooper JD, Belichenko PV, Salehi A, Mobley WC. Trafficking the NGF signal: implications for normal and degenerating neurons Progress in brain research. 2004;146:3-23.
Lee JH, Lau KF, Perkinton MS, Standen CL, Shemilt SJ, Mercken L, Cooper JD, McLoughlin DM, Miller CC. 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.
Cooper JD. Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis. Current opinion in neurology. 2003;16(2):121-8.
Heine C, Tyynelä J, Cooper JD, Palmer DN, Elleder M. et al. Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues The Biochemical journal. 2003;376(Pt 2):369-76.
Kesavapany S, Lau KF, Ackerley S, Banner SJ, Shemilt SJ, Cooper JD, Leigh PN, Shaw CE, McLoughlin DM, Miller CC. 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.
Kesavapany S, Banner SJ, Lau KF, Shaw CE, Miller CC, Cooper JD, McLoughlin DM. . Expression of the Fe65 adapter protein in adult and developing mouse brain. Neuroscience. 2002;115(3):951-60.
Chattopadhyay S, Ito M, Cooper JD, Brooks AI, Curran TM, Powers JM, Pearce DA. An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease Human molecular genetics. 2002;11(12):1421-31.
Cooper JD, Salehi A, Delcroix JD, Howe CL, Belichenko PV, Chua-Couzens J, Kilbridge JF, Carlson EJ, Epstein CJ, Mobley WC. 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.
Cooper JD, Mobley WC. Neurotrophic factors as potential therapeutic agents in neuronal ceroid lipofuscinosis. Advances in genetics. . 2001;45:169-82.
Cooper JD, Messer A, Feng AK, Chua-Couzens J, Mobley WC. 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.
Mitchison HM, Bernard DJ, Greene ND, Cooper JD, Junaid MA, Pullarkat RK, de Vos N, Breuning MH, Owens JW, Mobley WC, Gardiner RM, Lake BD, Taschner PE, Nussbaum RL. Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected] Neurobiology of disease. 1999;6(5):321-34.
Panni MK, Cooper JD, Sofroniew MV. Ganglioside GM1 potentiates NGF action on axotomised medial septal cholinergic neurons Brain research. 1998;812(1-2):76-80.
Yeo TT, Chua-Couzens J, Butcher LL, Bredesen DE, Cooper JD, Valletta JS, Mobley WC, Longo FM. 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.
Cooper JD, Sofroniew MV. Increased vulnerability of septal cholinergic neurons to partial loss of target neurons in aged rats Neuroscience. 1996;75(1):29-35.
Cooper JD, Skepper JN, Berzaghi MD, Lindholm D, Sofroniew MV. 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.
De Lacalle S, Cooper JD, Svendsen CN, Dunnett SB, Sofroniew MV. Reduced retrograde labelling with fluorescent tracer accompanies neuronal atrophy of basal forebrain cholinergic neurons in aged rats Neuroscience. 1996;75(1):19-27.
Michaelidis TM, Sendtner M, Cooper JD, Airaksinen MS, Holtmann B, Meyer M, Thoenen H. Inactivation of bcl-2 results in progressive degeneration of motoneurons, sympathetic and sensory neurons during early postnatal development Neuron. 1996;17(1):75-89.
Heisenberg CP, Cooper JD, Berke J, Sofroniew MV. NMDA potentiates NGF-induced sprouting of septal cholinergic fibres Neuroreport. 1994;5(4):413-6.
Cooper JD, Lindholm D, Sofroniew MV. 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.
Sofroniew MV, Cooper JD, Svendsen CN, Crossman P, Ip NY, Lindsay RM, Zafra F, Lindholm D. 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.
Svendsen CN, Cooper JD, Sofroniew MV. Trophic factor effects on septal cholinergic neurons. Annals of the New York Academy of Sciences. 1991;640:91-4.
Cornwall J, Cooper JD, Phillipson OT. Projections to the rostral reticular thalamic nucleus in the rat Experimental brain research. 1990;80(1):157-71.
Cornwall J, Cooper JD, Phillipson OT. Afferent and efferent connections of the laterodorsal tegmental nucleus in the rat. Brain research bulletin 1990;25(2):271-84.
Cooper JD, Payne JN, Horobin RW. Accurate counting of neurons in frozen sections: some necessary precautions. Journal of anatomy Journal of anatomy. 1988;80(1):13-21.
Payne JN, Cooper JD, MacKeown ST, Horobin RW. A temperature controlled chamber to allow observation and measurement of uptake of fluorochromes into live cells Journal of microscopy. 1987;14(3):329-35.
Hemanth R Nelvagal, Jenny Lange, Keigo Takahashi, Marta A Tarczyluk-Wells, Jonathan D Cooper. Pathomechanisms in the Neuronal Ceroid Lipofuscinoses BBA Molecular Basis of Disease.