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H. Nelvagal · SciRah

Researcher profile · Semantic Scholar

H. Nelvagal

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H. Nelvagal — researcher profile on SciRah

h-index: 16
Works: 50
Citations: 1,112

Most-cited papers

mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases
M. Palmieri, Rituraj Pal, H. Nelvagal, P. Lotfi +16 more
2017Nature Communications479 citations Open access
Semantic Scholar
Towards a new understanding of NCL pathogenesis.
J. Cooper, Marta A Tarczyluk, H. Nelvagal
2015Biochimica et Biophysica Acta81 citations Open access
Semantic Scholar
Pathomechanisms in the neuronal ceroid lipofuscinoses.
H. Nelvagal, Jenny Lange, Keigo Takahashi, Marta Tarczyluk-Wells +1 more
2020Biochimica et Biophysica Acta - Molecular Basis of Disease70 citations Open access
Semantic Scholar
Synergistic effects of treating the spinal cord and brain in CLN1 disease
Charles Shyng, H. Nelvagal, Joshua T. Dearborn, J. Tyynelä +3 more
2017Proceedings of the National Academy of Sciences of the United States of America65 citations Open access
Semantic Scholar
Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.
J. Groh, Thomas G. Kühl, C. Ip, H. Nelvagal +6 more
2013Brain : a journal of neurology55 citations Open access
Semantic Scholar
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
D. Sondhi, E. Scott, Alvin Chen, N. Hackett +7 more
2014Human Gene Therapy44 citations Open access
Semantic Scholar
Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.
Jui-Yun Lu, H. Nelvagal, Ling-Ling Wang, Shari G. Birnbaum +2 more
2015Molecular Genetics and Metabolism43 citations
Semantic Scholar
Neuronal deletion of the circadian clock gene Bmal1 induces cell-autonomous dopaminergic neurodegeneration
Michael K. Kanan, Patrick W. Sheehan, Jessica N. Haines, Pedro G. Gomez +10 more
2023JCI Insight35 citations Open access
Semantic Scholar
Spinal manifestations of CLN1 disease start during the early postnatal period
H. Nelvagal, Joshua T. Dearborn, J. Ostergaard, M. Sands +1 more
2020Neuropathology and Applied Neurobiology27 citations Open access
Semantic Scholar
Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures
Joshua T. Dearborn, H. Nelvagal, N. Rensing, Keigo Takahashi +5 more
2022Scientific Reports25 citations Open access
Semantic Scholar
Glial Dysfunction and Its Contribution to the Pathogenesis of the Neuronal Ceroid Lipofuscinoses
Keigo Takahashi, H. Nelvagal, Jenny Lange, J. Cooper
2022Frontiers in Neurology21 citations Open access
Semantic Scholar
Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor
M. Babcock, Christina R. Mikulka, Bing Wang, S. Chandriani +15 more
2021Scientific Reports20 citations Open access
Semantic Scholar
Gene therapy ameliorates bowel dysmotility and enteric neuron degeneration and extends survival in lysosomal storage disorder mouse models
Ewa A. Ziółkowska, Matthew J. Jansen, Letitia L. Williams, Sophie H. Wang +18 more
2025Science Translational Medicine19 citations
Semantic Scholar
Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice
K. White, H. Nelvagal, Timothy A. Poole, B. Lu +11 more
2021Molecular Therapy: Methods & Clinical Development18 citations Open access
Semantic Scholar
A Novel Small NPC1 Promoter Enhances AAV-Mediated Gene Therapy in Mouse Models of Niemann–Pick Type C1 Disease
M. Hughes, H. Nelvagal, Oliver Coombe-Tennant, David Smith +5 more
2023Cells16 citations Open access
Semantic Scholar
Gene therapy ameliorates spontaneous seizures associated with cortical neuron loss in a Cln2R207X mouse model
Keigo Takahashi, Elizabeth M. Eultgen, Sophie H. Wang, N. Rensing +7 more
2023Journal of Clinical Investigation16 citations Open access
Semantic Scholar
Top-down and bottom-up propagation of disease in the neuronal ceroid lipofuscinoses
J. Ostergaard, H. Nelvagal, J. Cooper
2022Frontiers in Neurology14 citations Open access
Semantic Scholar
Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease
H. Nelvagal, Maica Llavero Hurtado, S. Eaton, Rachel A. Kline +4 more
2020Scientific Reports14 citations Open access
Semantic Scholar
Cross-species efficacy of enzyme replacement therapy for CLN1 disease in mice and sheep
H. Nelvagal, S. Eaton, Sophie H. Wang, Elizabeth M. Eultgen +26 more
2022Journal of Clinical Investigation13 citations Open access
Semantic Scholar
Comparison of different promoters to improve AAV vector-mediated gene therapy for neuronopathic Gaucher disease
Giulia Massaro, Amy Geard, H. Nelvagal, Katrina Gore +3 more
2024Human Molecular Genetics7 citations Open access
Semantic Scholar
Tralesinidase Alfa Enzyme Replacement Therapy Prevents Disease Manifestations in a Canine Model of Mucopolysaccharidosis Type IIIB
N. Ellinwood, Bethann N. Valentine, A. Hess, J. Jens +33 more
2022Journal of Pharmacology and Experimental Therapeutics7 citations Open access
Semantic Scholar
Translating preclinical models of neuronal ceroid lipofuscinosis: progress and prospects
H. Nelvagal, J. Cooper
20176 citations
Semantic Scholar
Molecular and cellular signatures differentiate Parkinson’s disease from Parkinson’s disease with dementia
Á. Fairbrother-Browne, M. Grant-Peters, J. Brenton, H. Nelvagal +18 more
2025bioRxiv4 citations Open access
Semantic Scholar
Quantifying storage material accumulation in tissue sections.
J. Cooper, Helen R. Brooks, H. Nelvagal
2015Methods in Cell Biology3 citations
Semantic Scholar
GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice
Keigo Takahashi, N. Rensing, Elizabeth M. Eultgen, Letitia L. Williams +10 more
2025JCI Insight2 citations Open access
Semantic Scholar
Recombinant NAGLU-IGF2 prevents physical and neurological disease and improves survival in Sanfilippo B syndrome
S. Le, Shih-Hsin Kan, Marie S. Roberts, Joshua T. Dearborn +16 more
2021bioRxiv2 citations Open access
Semantic Scholar
Progress toward Fulfilling the Potential of Immunomodulation in Childhood Neurodegeneration?
J. Cooper, H. Nelvagal
2017Molecular Therapy2 citations Open access
Semantic Scholar
Progressive neuropathology in the spinal cord of a mouse model of CLN1 disease and assessing the efficacy of intrathecal enzyme replacement therapy (ERT)
J. Cooper, J. Dmytrus, Sarmi Sri, Joshua T. Dearborn +5 more
20162 citations
Semantic Scholar
A molecular map of the living human brain from quantitative MRI
M. Grant-Peters, G. Thomas, Daan van Kruining, Louise A. Huuki-Myers +21 more
2026bioRxiv1 citations Open access
Semantic Scholar
Data-driven modelling of tau pathology reveals distinct progressive supranuclear palsy subtypes
Patrick W. Cullinane, J. Parmera, H. Nelvagal, Toby Curless +23 more
2025medRxiv1 citations Open access
Semantic Scholar
Cellular trafficking and fate mapping of cells within the nervous system after in utero hematopoietic cell transplantation
Matthew T. Grant, H. Nelvagal, Maria E. Tecos, A. Hamed +3 more
2024Communications Biology1 citations Open access
Semantic Scholar
Cortical interneuron loss and seizure generation as novel clinically relevant disease phenotypes in Cln2R207X mice
Keigo Takahashi, Elizabeth M. Eultgen, Sophie H. Wang, N. Rensing +5 more
2022bioRxiv1 citations Open access
Semantic Scholar
Devising effective enzyme replacement therapy for infantile onset neuronal ceroid lipofuscinosis (CLN1 disease)
J. Cooper, A. C. Puhl, Sophie H. Wang, Elizabeth M. Eultgen +4 more
20211 citations
Semantic Scholar
Immunostaining Methods for Lysosomal Storage Disorder Research.
Ewa A. Ziółkowska, Sophie H. Wang, H. Nelvagal, Jonathan D. Cooper
2026Methods in molecular biology0 citations
Semantic Scholar
Cause, consequence, or synergy: the role of T-cell infiltration across neurodegenerative diseases.
Patrick W. Cullinane, H. Nelvagal, Cyrus Wong, Toby Curless +11 more
2026Brain : a journal of neurology0 citations
Semantic Scholar
Quantitative pathology and APOE genotype reveal dementia risk and progression in Lewy body disease
H. Nelvagal, Nancy Chiraki, Toby Curless, Patrick W. Cullinane +15 more
2025medRxiv0 citations Open access
Semantic Scholar
Treatment of dysphagia associated pathologies in CLN3 deficient mice via gene therapy
Ewa A. Ziółkowska, Letitia L. Williams, Elizabeth M. Eultgen, A. Jablonka-Shariff +5 more
2025Molecular Genetics and Metabolism0 citations
Semantic Scholar
Limited therapeutic efficacy of N-acetyl-L-leucine in a mouse model of CLN1 disease
Ewa A. Ziółkowska, Nicole A. Pagán Torres, Hsintsung Chen, Letitia L. Williams +7 more
2025Scientific Reports0 citations Open access
Semantic Scholar
GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice
Keigo Takahashi, N. Rensing, Elizabeth M. Eultgen, Sophie H. Wang +9 more
2024bioRxiv0 citations
Semantic Scholar
Neuronal deletion of the circadian clock gene Bmal1 induces cell-autonomous dopaminergic neurodegeneration
Michael Kanan, Patrick W. Sheehan, Jessica N. Haines, Pedro G. Gomez +10 more
2024JCI Insight0 citations
Semantic Scholar
Spontaneous seizures associated with cortical interneuron loss in mice are ameliorated via gene therapy
Keigo Takahashi, Elizabeth M. Eultgen, Sophie H. Wang, N. Rensing +7 more
2023Molecular Genetics and Metabolism0 citations
Semantic Scholar
Title: Bowel dysmotility and enteric neuron degeneration in lysosomal storage disease mice is prevented by gene therapy Short title: Treatment of enteric disease by gene therapy
Elizabeth M. Eultgen, Keigo, Takahashi, H. Nelvagal +11 more
20230 citations
Semantic Scholar
Efficacy of recombinant human PPT1 enzyme replacement therapy in mouse and sheep models of CLN1 disease
H. Nelvagal, S. Eaton, Sophie H. Wang, Elizabeth M. Eultgen +13 more
2022Molecular Genetics and Metabolism0 citations
Semantic Scholar
Spinal cord pathology in murine Sanfilippo syndrome type B
S. Le, Alexander Sorensen, K. Chintalapati, Shih-Hsin Kan +3 more
2022Molecular Genetics and Metabolism0 citations
Semantic Scholar
Amelioration of enteric nervous system defects via gene therapy in CLN1 disease mice
J. Cooper, Sophie H. Wang, Letitia L. Williams, Elizabeth M. Eultgen +7 more
2022Molecular Genetics and Metabolism0 citations
Semantic Scholar
Subretinal injection of RGX-381 to cynomolgus monkeys leads to supraphysiological levels of TPP1 in the eye
N. Buss, E. Budzynski, Lin Yang, Sunjung Kim +9 more
20210 citations
Semantic Scholar
An update on the progress of preclinical models for guiding therapeutic management of neuronal ceroid lipofuscinosis
H. Nelvagal, J. Cooper
20190 citations
Semantic Scholar
Early gait abnormalities relate to brainstem and spinal cord pathology in CLN1 disease
H. Nelvagal, Charles Shyng, M. Sands, J. Cooper
20180 citations
Semantic Scholar
Spinal cord pathology in CLN1 disease : a novel therapeutic target
H. Nelvagal
20170 citations
Semantic Scholar
27. Therapeutic Efficacy of Intracranial and Intrathecal AAV2/9-PPT1 in Infantile Batten Disease
Charles Shyng, H. Nelvagal, Joshua T. Dearborn, J. Cooper +1 more
2016Molecular Therapy0 citations Open access
Semantic Scholar

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Elizabeth M. Eultgen×13
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Joshua T. Dearborn×12
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Keigo Takahashi

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Sophie H. Wang×12

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Jonathan D. Cooper×9

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N. Rensing — researcher profile on SciRah

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