Jon J Brudvig - Publications

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20 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2024 Schulz A, Patel N, Brudvig JJ, Stehr F, Weimer JM, Augustine EF. The parent and family impact of CLN3 disease: an observational survey-based study. Orphanet Journal of Rare Diseases. 19: 125. PMID 38500130 DOI: 10.1186/s13023-024-03119-8  0.646
2023 Leppert HG, Anderson JT, Timm KJ, Davoli C, Pratt MA, Booth CD, White KA, Rechtzigel MJ, Meyerink BL, Johnson TB, Brudvig JJ, Weimer JM. Sortilin inhibition treats multiple neurodegenerative lysosomal storage disorders. Biorxiv : the Preprint Server For Biology. PMID 37790379 DOI: 10.1101/2023.09.22.559064  0.669
2023 Swier VJ, White KA, Johnson TB, Wang X, Han J, Pearce DA, Singh R, Drack AV, Pfeifer W, Rogers CS, Brudvig JJ, Weimer JM. A novel porcine model of CLN3 Batten disease recapitulates clinical phenotypes. Disease Models & Mechanisms. PMID 37305926 DOI: 10.1242/dmm.050038  0.676
2023 Johnson TB, Brudvig JJ, Likhite S, Pratt MA, White KA, Cain JT, Booth CD, Timm DJ, Davis SS, Meyerink B, Pineda R, Dennys-Rivers C, Kaspar BK, Meyer K, Weimer JM. Early postnatal administration of an AAV9 gene therapy is safe and efficacious in CLN3 disease. Frontiers in Genetics. 14: 1118649. PMID 37035740 DOI: 10.3389/fgene.2023.1118649  0.762
2022 Brudvig JJ, Swier VJ, Johnson TB, Cain JC, Pratt M, Rechtzigel M, Leppert H, Dang Do AN, Porter FD, Weimer JM. Glycerophosphoinositol is Elevated in Blood Samples From pigs, Mice, and CLN3-Affected Individuals. Biomarker Insights. 17: 11772719221107765. PMID 36212622 DOI: 10.1177/11772719221107765  0.659
2022 Swier VJ, White KA, Johnson TB, Sieren JC, Johnson HJ, Knoernschild K, Wang X, Rohret FA, Rogers CS, Pearce DA, Brudvig JJ, Weimer JM. A Novel Porcine Model of CLN2 Batten Disease that Recapitulates Patient Phenotypes. Neurotherapeutics : the Journal of the American Society For Experimental Neurotherapeutics. PMID 36100791 DOI: 10.1007/s13311-022-01296-7  0.653
2022 Rechtzigel MJ, Meyerink BL, Leppert H, Johnson TB, Cain JT, Ferrandino G, May DG, Roux KJ, Brudvig JJ, Weimer JM. Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment. Frontiers in Neuroscience. 16: 834780. PMID 35692423 DOI: 10.3389/fnins.2022.834780  0.752
2022 Brudvig JJ, Weimer JM. CLN7 gene therapy: hope for an ultra-rare condition. The Journal of Clinical Investigation. 132. PMID 35229731 DOI: 10.1172/JCI157820  0.654
2022 Muthusamy N, Williams TI, O'Toole R, Brudvig JJ, Adler KB, Weimer JM, Muddiman DC, Ghashghaei HT. Phosphorylation-dependent proteome of Marcks in ependyma during aging and behavioral homeostasis in the mouse forebrain. Geroscience. PMID 35075585 DOI: 10.1007/s11357-022-00517-3  0.578
2021 Lu CL, Ortmeier S, Brudvig J, Moretti T, Cain J, Boyadjiev SA, Weimer JM, Kim J. Collagen has a unique SEC24 preference for efficient export from the endoplasmic reticulum. Traffic (Copenhagen, Denmark). PMID 34761479 DOI: 10.1111/tra.12826  0.714
2021 Brudvig JJ, Weimer JM. On the cusp of cures: Breakthroughs in Batten disease research. Current Opinion in Neurobiology. 72: 48-54. PMID 34571324 DOI: 10.1016/j.conb.2021.08.003  0.67
2021 Selvan N, Mehta N, Venkateswaran S, Brignol N, Graziano M, Sheikh MO, McAnany Y, Hung F, Madrid M, Krampetz R, Siano N, Mehta A, Brudvig J, Gotschall R, Weimer JM, et al. Endolysosomal N-glycan processing is critical to attain the most active form of the enzyme acid alpha-glucosidase. The Journal of Biological Chemistry. 100769. PMID 33971197 DOI: 10.1016/j.jbc.2021.100769  0.579
2021 White KA, Nelvagal HR, Poole TA, Lu B, Johnson TB, Davis S, Pratt MA, Brudvig J, Assis AB, Likhite S, Meyer K, Kaspar BK, Cooper JD, Wang S, Weimer JM. Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice. Molecular Therapy. Methods & Clinical Development. 20: 497-507. PMID 33665223 DOI: 10.1016/j.omtm.2020.12.014  0.645
2020 Johnson TB, White KA, Brudvig JJ, Cain JT, Langin L, Pratt MA, Booth CD, Timm DJ, Davis SS, Meyerink B, Likhite S, Meyer K, Weimer JM. AAV9 Gene Therapy Increases Lifespan and Treats Pathological and Behavioral Abnormalities in a Mouse Model of CLN8-Batten Disease. Molecular Therapy : the Journal of the American Society of Gene Therapy. PMID 33010819 DOI: 10.1016/j.ymthe.2020.09.033  0.773
2020 Johnson TB, Brudvig JJ, Lehtimäki KK, Cain JT, White KA, Bragge T, Rytkönen J, Huhtala T, Timm D, Vihma M, Puoliväli JT, Poutiainen P, Nurmi A, Weimer JM. A multimodal approach to identify clinically relevant biomarkers to comprehensively monitor disease progression in a mouse model of pediatric neurodegenerative disease. Progress in Neurobiology. 101789. PMID 32198061 DOI: 10.1016/J.Pneurobio.2020.101789  0.77
2019 Cain JT, Johnson TB, White K, Meyerink B, Staton C, Davis S, Sturdevant D, Bragge T, Huhtala T, Brudvig J, Rytkonen J, Lehtimaki K, Nurmi A, Nurmi A, Weimer J. Identifying a biomarker signature for Batten disease Molecular Genetics and Metabolism. 126: S35-S36. DOI: 10.1016/J.Ymgme.2018.12.070  0.754
2018 Brudvig JJ, Cain JT, Sears RM, Schmidt-Grimminger GG, Wittchen ES, Adler KB, Ghashghaei HT, Weimer JM. MARCKS regulates neuritogenesis and interacts with a CDC42 signaling network. Scientific Reports. 8: 13278. PMID 30185885 DOI: 10.1038/S41598-018-31578-0  0.725
2018 Brudvig JJ, Cain JT, Schmidt-Grimminger GG, Stumpo DJ, Roux KJ, Blackshear PJ, Weimer JM. MARCKS Is Necessary for Netrin-DCC Signaling and Corpus Callosum Formation. Molecular Neurobiology. PMID 29546593 DOI: 10.1007/S12035-018-0990-3  0.709
2015 Brudvig JJ, Weimer JM. X MARCKS the spot: myristoylated alanine-rich C kinase substrate in neuronal function and disease. Frontiers in Cellular Neuroscience. 9: 407. PMID 26528135 DOI: 10.3389/Fncel.2015.00407  0.624
2015 Brudvig J, Cain J. KIS: synaptic plasticity's missing molecular link? The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 35: 2839-41. PMID 25698723 DOI: 10.1523/Jneurosci.5082-14.2015  0.653
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