Jonathan D. Cooper

MedicineBiochemistry, Genetics and Molecular Biology

Pilot corpus only

This score is computed over theSindex pilot corpus and does not cover the full scientific literature. Scores are relative to papers we have ingested — papers, authors, and institutions outside the pilot are not represented. Methodology.

Top 100%percentile

0Author DataRank

Indexed papers

2in pilot corpus

datarank_citation_only_1hop_v6· scope data_onlyMethodology

Why this DataRank?

An author's DataRank is the sum of the DataRanks of all 2 indexed papers attributed to them. A prolific author with many moderate-impact papers can outrank one with a single high-impact paper.

Author scores recompute whenever paper DataRanks are refreshed, so this number lags the underlying paper scores by at most one batch run.

Read the full methodology →

Papers

Driven by 26 papers. Top paper: “Absence of p75NTRCauses Increased Basal Forebrain Cholinergic Neuron Size, Choline Acetyltransferase Activity, and Target Innervation”.

Absence of p75NTRCauses Increased Basal Forebrain Cholinergic Neuron Size, Choline Acetyltransferase Activity, and Target Innervation

The Journal of Neuroscience(1997)10.1523/jneurosci.17-20-07594.1997

N/A

0.800DataRank · unranked

206 citations

Jane Chua-Couzens, Larry L. Butcher, Dale E. Bredesen, Jonathan D. Cooper, Janice S. Valletta +3 more

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

Human Gene Therapy(2014)10.1089/hum.2012.253

N/A

0.571DataRank · unranked

44 citations

Emma C. Scott, Alvin Chen, Neil R. Hackett, Andrew M.S. Wong, Agnieszka Kubiak +6 more

Spinal manifestations of CLN1 disease start during the early postnatal period

Neuropathology and Applied Neurobiology(2020)10.1111/nan.12658

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0.505DataRank · unranked

28 citations

Joshua T. Dearborn, John R. Østergaard, Mark S. Sands, Jonathan D. Cooper, Hemanth R. Nelvagal

Cross-species efficacy of enzyme replacement therapy for CLN1 disease in mice and sheep

Journal of Clinical Investigation(2022)10.1172/jci163107

N/A

0.449DataRank · unranked

19 citations

Samantha L. Eaton, Sophie H. Wang, Elizabeth M. Eultgen, Keigo Takahashi, Steven Q. Le +25 more

Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice

Molecular Therapy — Methods & Clinical Development(2021)10.1016/j.omtm.2020.12.014

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0.449DataRank · unranked

19 citations

Hemanth R. Nelvagal, Timothy A. Poole, Bin Lü, Tyler B. Johnson, Samantha S. Davis +10 more

Neuronal ceroid lipofuscinosis: underlying mechanisms and emerging therapeutic targets

Nature Reviews Neurology(2025)10.1038/s41582-025-01132-4

N/A

0.342DataRank · unranked

8 citations

Keigo Takahashi, Patricia Dickson, Marco Sardiello, Mark S. Sands, Jonathan D. Cooper +1 more

Tralesinidase Alfa Enzyme Replacement Therapy Prevents Disease Manifestations in a Canine Model of Mucopolysaccharidosis Type IIIB

Journal of Pharmacology and Experimental Therapeutics(2022)10.1124/jpet.122.001119

N/A

0.339DataRank · unranked

7 citations

Bethann Valentine, Andrew S. Hess, Jackie K. Jens, Elizabeth Snella, Maryam Jamil +32 more

Enteric nervous system degeneration in human and murine CLN3 disease, is ameliorated by gene therapy in mice

Acta Neuropathologica Communications(2025)10.1186/s40478-025-02205-7

N/A

0.104DataRank · unranked

1 citations

Letitia L. Williams, Matthew J. Jansen, S. L. Wang, Elizabeth M. Eultgen, Jaiprakash Sharma +7 more

Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures

bioRxiv (Cold Spring Harbor Laboratory)(2022)10.1101/2022.03.07.483344

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0DataRank · unranked

1 citations

Hemanth R. Nelvagal, Nicholas Rensing, Stephanie M. Hughes, Thomas M. Wishart, Jonathan D. Cooper +3 more

Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease

Scientific Reports(2020)10.1038/s41598-020-72075-7

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0DataRank · unranked

15 citations

Maica Llavero Hurtado, Samantha L. Eaton, Rachel Kline, Douglas J. Lamont, Mark S. Sands +3 more

Neuronal deletion of the circadian clock gene Bmal1 induces cell-autonomous dopaminergic neurodegeneration

JCI Insight(2023)10.1172/jci.insight.162771

N/A

0DataRank · unranked

29 citations

Patrick W. Sheehan, Jessica N. Haines, P. Gómez, Adya Dhuler, Collin J. Nadarajah +9 more

Gene therapy ameliorates spontaneous seizures associated with cortical neuron loss in a Cln2R207X mouse model

Journal of Clinical Investigation(2023)10.1172/jci165908

N/A

0DataRank · unranked

18 citations

Elizabeth M. Eultgen, Sophie H. Wang, Nicholas Rensing, Hemanth R. Nelvagal, Joshua T. Dearborn +6 more

Bowel dysmotility and enteric neuron degeneration in lysosomal storage disease mice is prevented by gene therapy

bioRxiv (Cold Spring Harbor Laboratory)(2023)10.1101/2023.05.26.542524

N/A

0DataRank · unranked

2 citations

Letitia L. Williams, Sophie H. Wang, Elizabeth M. Eultgen, Keigo Takahashi, Hemanth R. Nelvagal +12 more

A mouse mutant deficient in both neuronal ceroid lipofuscinosis‐associated proteins CLN3 and TPP1

Journal of Inherited Metabolic Disease(2023)10.1002/jimd.12619

N/A

0DataRank · unranked

0 citations

Whitney Banach‐Petrosky, Katherine E. Larrimore, Yuliya Nemtsova, Jennifer A. Wiseman, Allison R. Najafi +6 more

GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice

bioRxiv (Cold Spring Harbor Laboratory)(2024)10.1101/2024.03.29.587276

N/A

0DataRank · unranked

0 citations

Nicholas Rensing, Elizabeth M. Eultgen, Sophie H. Wang, Hemanth R. Nelvagal, Steven Q. Le +8 more

Gene therapy ameliorates bowel dysmotility and enteric neuron degeneration and extends survival in lysosomal storage disorder mouse models

Science Translational Medicine(2025)10.1126/scitranslmed.adj1445

N/A

0DataRank · unranked

17 citations

Matthew J. Jansen, Letitia L. Williams, Sophie H. Wang, Elizabeth M. Eultgen, Keigo Takahashi +17 more

Gene therapy ameliorates neuromuscular pathology in CLN3 disease

Acta Neuropathologica Communications(2025)10.1186/s40478-025-02059-z

N/A

0DataRank · unranked

6 citations

Albina Jablonka‐Shariff, Letitia L. Williams, Matthew J. Jansen, Sophie H. Wang, Elizabeth M. Eultgen +10 more

Limited therapeutic efficacy of N-acetyl-L-leucine in a mouse model of CLN1 disease

Scientific Reports(2025)10.1038/s41598-025-32984-x

N/A

0DataRank · unranked

0 citations

Nicole A. Pagán Torres, Hsintsung Chen, Letitia L. Williams, Elizabeth M. Eultgen, Agnieszka Nowacka +6 more

Cortical interneuron loss and seizure generation as novel clinically relevant disease phenotypes in Cln2 R207X mice

bioRxiv (Cold Spring Harbor Laboratory)(2022)10.1101/2022.03.11.483984

N/A

0DataRank · unranked

0 citations

Elizabeth M. Eultgen, Sophie H. Wang, Nicholas Rensing, Hemanth R. Nelvagal, Joshua T. Dearborn +4 more

Neuropathology of murine Sanfilippo D syndrome

Molecular Genetics and Metabolism(2021)10.1016/j.ymgme.2021.11.010

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0DataRank · unranked

8 citations

Steven Q. Le, Shih‐hsin Kan, Matthew J. Jansen, Patricia Dickson, Jonathan D. Cooper +1 more

Biochemical evaluation of intracerebroventricular rhNAGLU-IGF2 enzyme replacement therapy in neonatal mice with Sanfilippo B syndrome

Molecular Genetics and Metabolism(2021)10.1016/j.ymgme.2021.03.013

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0DataRank · unranked

7 citations

Ibrahim Elsharkawi, Steven Q. Le, Heather Prill, Linley Mangini, Jonathan D. Cooper +5 more

Recombinant NAGLU-IGF2 prevents physical and neurological disease and improves survival in Sanfilippo B syndrome

bioRxiv (Cold Spring Harbor Laboratory)(2021)10.1101/2021.08.06.455469

N/A

0DataRank · unranked

1 citations

Shih‐hsin Kan, Marie S. Roberts, Joshua T. Dearborn, Rui Wang, Shan Li +15 more

Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures

Scientific Reports(2022)10.1038/s41598-022-15134-5

N/A

0DataRank · unranked

25 citations

Hemanth R. Nelvagal, Nicholas Rensing, Keigo Takahashi, Stephanie M. Hughes, Thomas M. Wishart +4 more

Glial Dysfunction and Its Contribution to the Pathogenesis of the Neuronal Ceroid Lipofuscinoses

Frontiers in Neurology(2022)10.3389/fneur.2022.886567

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0DataRank · unranked

23 citations

Hemanth R. Nelvagal, Jenny Lange, Jonathan D. Cooper, Keigo Takahashi

Modelling Neurological Diseases in Large Animals: Criteria for Model Selection and Clinical Assessment

Cells(2022)10.3390/cells11172641

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0DataRank · unranked

9 citations

Fraser Murdoch, Nina M. Rzechorzek, Gerard Thompson, Claudia Hartley, B. Blacklock +13 more

Brain transplantation of genetically corrected Sanfilippo type B neural stem cells induces partial cross-correction of the disease

Molecular Therapy — Methods & Clinical Development(2022)10.1016/j.omtm.2022.10.013

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0DataRank · unranked

5 citations

Don Clarke, Shih‐hsin Kan, Steven Q. Le, Valentina Sanghez, Anna Luzzi +6 more