| mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases M Palmieri, R Pal, HR Nelvagal, P Lotfi, GR Stinnett, ML Seymour, ... Nature communications 8 (1), 14338, 2017 | 397 | 2017 |
| Towards a new understanding of NCL pathogenesis JD Cooper, MA Tarczyluk, HR Nelvagal Biochimica Et Biophysica Acta (BBA)-Molecular Basis of Disease 1852 (10 …, 2015 | 84 | 2015 |
| Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis J Groh, TG Kühl, CW Ip, HR Nelvagal, S Sri, S Duckett, M Mirza, ... Brain 136 (4), 1083-1101, 2013 | 65 | 2013 |
| Pathomechanisms in the neuronal ceroid lipofuscinoses HR Nelvagal, J Lange, K Takahashi, MA Tarczyluk-Wells, JD Cooper Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1866 (9), 165570, 2020 | 59 | 2020 |
| Synergistic effects of treating the spinal cord and brain in CLN1 disease C Shyng, HR Nelvagal, JT Dearborn, J Tyynelä, RE Schmidt, MS Sands, ... Proceedings of the National Academy of Sciences 114 (29), E5920-E5929, 2017 | 56 | 2017 |
| 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 … D Sondhi, EC Scott, A Chen, NR Hackett, AMS Wong, A Kubiak, ... Human gene therapy 25 (3), 223-239, 2014 | 44 | 2014 |
| Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis JY Lu, HR Nelvagal, L Wang, SG Birnbaum, JD Cooper, SL Hofmann Molecular genetics and metabolism 116 (1-2), 98-105, 2015 | 42 | 2015 |
| Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice KA White, HR Nelvagal, TA Poole, B Lu, TB Johnson, S Davis, MA Pratt, ... Molecular Therapy-Methods & Clinical Development 20, 497-507, 2021 | 20 | 2021 |
| Correction: Corrigendum: mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases M Palmieri, R Pal, HR Nelvagal, P Lotfi, GR Stinnett, ML Seymour, ... Nature communications 8 (1), 15793, 2017 | 20 | 2017 |
| Spinal manifestations of CLN1 disease start during the early postnatal period HR Nelvagal, JT Dearborn, JR Ostergaard, MS Sands, JD Cooper Neuropathology and applied neurobiology 47 (2), 251-267, 2021 | 15 | 2021 |
| Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor MC Babcock, CR Mikulka, B Wang, S Chandriani, S Chandra, Y Xu, ... Scientific reports 11 (1), 14486, 2021 | 13 | 2021 |
| Glial dysfunction and its contribution to the pathogenesis of the neuronal ceroid lipofuscinoses K Takahashi, HR Nelvagal, J Lange, JD Cooper Frontiers in Neurology 13, 886567, 2022 | 11 | 2022 |
| mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases. Nat Commun. 2017; 8: 14338 M Palmieri, R Pal, HR Nelvagal, P Lotfi, GR Stinnett, ML Seymour, ... | 11 | |
| Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease HR Nelvagal, ML Hurtado, SL Eaton, RA Kline, DJ Lamont, MS Sands, ... Scientific reports 10 (1), 15157, 2020 | 10 | 2020 |
| Cross-species efficacy of enzyme replacement therapy for CLN1 disease in mice and sheep HR Nelvagal, SL Eaton, SH Wang, EM Eultgen, K Takahashi, SQ Le, ... The Journal of Clinical Investigation 132 (20), 2022 | 6 | 2022 |
| Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures JT Dearborn, HR Nelvagal, NR Rensing, K Takahashi, SM Hughes, ... Scientific reports 12 (1), 11286, 2022 | 6 | 2022 |
| Translating preclinical models of neuronal ceroid lipofuscinosis: progress and prospects HR Nelvagal, JD Cooper Expert Opinion on Orphan Drugs 5 (9), 727-740, 2017 | 6 | 2017 |
| Tralesinidase alfa enzyme replacement therapy prevents disease manifestations in a canine model of mucopolysaccharidosis type IIIB NM Ellinwood, BN Valentine, AS Hess, JK Jens, EM Snella, M Jamil, ... Journal of Pharmacology and Experimental Therapeutics 382 (3), 277-286, 2022 | 5 | 2022 |
| Gene therapy ameliorates spontaneous seizures associated with cortical neuron loss in a Cln2R207X mouse model K Takahashi, EM Eultgen, SH Wang, NR Rensing, HR Nelvagal, ... The Journal of Clinical Investigation 133 (12), 2023 | 4 | 2023 |
| Neuronal deletion of the circadian clock gene Bmal1 induces cell-autonomous dopaminergic neurodegeneration MK Kanan, PW Sheehan, JN Haines, PG Gomez, A Dhuler, CJ Nadarajah, ... JCI insight 9 (2), 2024 | 2 | 2024 |
