ArticleActa neuropathologica2020
Shortening heparan sulfate chains prolongs survival and reduces parenchymal plaques in prion disease caused by mobile, ADAM10-cleaved prions.
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Article in Acta neuropathologica, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 1 of them a synthesis that pooled it.
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24citing papers in PubMed, 1 pooled it
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Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.
- Alpha-Synuclein Strain Variability in Body-First and Brain-First Synucleinopathies.Frontiers in aging neuroscience · 2022Pooled it
- Aquaporin-4 mislocalization from astrocyte endfeet prolongs survival in a prion-cerebral amyloid angiopathy model.bioRxiv : the preprint server for biology · 2026Article
- PrPStem cell reports · 2026Article
- D178N prion protein mutation endows RML prions with new strain properties that do not mimic human genetic prion diseases.Acta neuropathologica · 2026Article
- Spontaneous generation of diverse recombinant prion strains: sulfated glycan cofactors facilitate strain emergence but do not determine specific strain properties.Acta neuropathologica communications · 2025Article
- Cofactors facilitate bona fide prion misfolding in vitro but are not necessary for the infectivity of recombinant murine prions.PLoS pathogens · 2025Article
- Cleavage site-directed antibodies reveal the prion protein in humans is shed by ADAM10 at Y226 and associates with misfolded protein deposits in neurodegenerative diseases.Acta neuropathologica · 2024Article
- A Protein Misfolding Shaking Amplification-based method for the spontaneous generation of hundreds of bona fide prions.Nature communications · 2024Article
- Creutzfeldt-Jakob disease and other prion diseases.Nature reviews. Disease primers · 2024Review
- Review
- Exploring the heterogeneity of osteosarcoma cell characteristics and metabolic states and their association with clinical prognosis.Frontiers in immunology · 2024Article
- Understanding the key features of the spontaneous formation of bona fide prions through a novel methodology that enables their swift and consistent generation.Acta neuropathologica communications · 2023Article
- Neuronal Ndst1 depletion accelerates prion protein clearance and slows neurodegeneration in prion infection.PLoS pathogens · 2023Article
- Inducing prion protein shedding as a neuroprotective and regenerative approach in pathological conditions of the brain: from theory to facts.Neural regeneration research · 2023Review
- Thiophene-Based Ligands: Design, Synthesis and Their Utilization for Optical Assignment of Polymorphic-Disease-Associated Protein Aggregates.Chembiochem : a European journal of chemical biology · 2023Review
- Diminished Neuronal ESCRT-0 Function Exacerbates AMPA Receptor Derangement and Accelerates Prion-Induced Neurodegeneration.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2023Article
- Anchorless risk or released benefit? An updated view on the ADAM10-mediated shedding of the prion protein.Cell and tissue research · 2023Review
- Pathogenic prion structures at high resolution.PLoS pathogens · 2022Article
- Ligands binding to the prion protein induce its proteolytic release with therapeutic potential in neurodegenerative proteinopathies.Science advances · 2021Article
- Distinct conformers of amyloid beta accumulate in the neocortex of patients with rapidly progressive Alzheimer's disease.The Journal of biological chemistry · 2021Article
Corrections and comments
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Authors and funding
18 authors at 5 institutions in 3 countries.
Patricia Aguilar-CalvoDepartment of Pathology, University of California, San Diego (UCSD), 9500 Gilman Dr., La Jolla, CA, 92093, USA.
Alejandro M SevillanoDepartment of Pathology, University of California, San Diego (UCSD), 9500 Gilman Dr., La Jolla, CA, 92093, USA.
Jaidev BapatDepartment of Pathology, University of California, San Diego (UCSD), 9500 Gilman Dr., La Jolla, CA, 92093, USA.
Katrin SoldauDepartment of Pathology, University of California, San Diego (UCSD), 9500 Gilman Dr., La Jolla, CA, 92093, USA.
Daniel R SandovalDepartment of Cellular and Molecular Medicine, University of California, San Diego (UCSD), La Jolla, CA, USA.
Hermann C AltmeppenInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany.
Luise LinsenmeierInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany.
Donald P PizzoDepartment of Pathology, University of California, San Diego (UCSD), 9500 Gilman Dr., La Jolla, CA, 92093, USA.
Michael D GeschwindDepartment of Neurology, Memory and Aging Center, University of California, San Francisco (UCSF), San Francisco, CA, USA.
Henry SanchezDepartment of Neurology, Memory and Aging Center, University of California, San Francisco (UCSF), San Francisco, CA, USA.
Brian S ApplebyDepartments of Pathology and Neurology, Case Western Reserve University, Cleveland, OH, USA.
Mark L CohenDepartments of Pathology and Neurology, Case Western Reserve University, Cleveland, OH, USA.
Jiri G SafarDepartments of Pathology and Neurology, Case Western Reserve University, Cleveland, OH, USA.
Steven D EdlandDepartment of Family Medicine and Public Health, University of California, San Diego (UCSD), La Jolla, CA, USA.
Markus GlatzelInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany.
K Peter R NilssonDepartment of Physics, Chemistry, and Biology, Linköping University, Linköping, Sweden.
Jeffrey D EskoDepartment of Cellular and Molecular Medicine, University of California, San Diego (UCSD), La Jolla, CA, USA.
Christina J SigurdsonDepartment of Pathology, University of California, San Diego (UCSD), 9500 Gilman Dr., La Jolla, CA, 92093, USA. csigurdson@ucsd.edu.ORCID http://orcid.org/0000-0002-1770-0965
University of California San Diego · USCase Western Reserve University · USUniversität Hamburg · DEUniversity of California, San Francisco · USLinköping University · SE
Funding
UC San Diego Clinical and Translational Research InstituteUL1TR001442 · NCATS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI FIRESTEIN, GARY S, HOGARTH, MICHAEL · 2015 to 2024
$88.3MUCSD Shiley-Marcos Alzheimer's Disease Research Center P30P30AG062429 · NIA · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI Subhojit Roy · 2019 to 2026
$34.9MPredicting progression of human prion diseaseR01AG031189 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GESCHWIND, MICHAEL D · 2008 to 2017
$5.7MMechanisms of Prion Aggregation and Species BarriersR01NS069566 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI ESKO, JEFFREY D, RAVITS, JOHN · 2011 to 2025
$5.7MTracking longitudinal change in presymptomatic genetic prion disease (TLC-Pre-gPrD)R01AG062562 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GESCHWIND, MICHAEL D · 2019 to 2023
$5.4MMechanisms of Prion SpreadR01NS076896 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI SIGURDSON, CHRISTINA · 2012 to 2021
$3.4MReplication mechanism of human prionsR01NS103848 · NINDS · CASE WESTERN RESERVE UNIVERSITY · PI SAFAR, JIRI G., SUREWICZ, WITOLD K · 2018 to 2022
$2.6MMechanisms of Prion Spread and Neuronal ToxicityR37NS076896 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI Christina Sigurdson · 2023 to 2026
$2.6MDrug Discovery for Multiple Hereditary ExostosesR01AR064202 · NIAMS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI ESKO, JEFFREY D · 2013 to 2017
$1.9MRole of heparan sulfate in neural cell vulnerability to prionsR00AG061251 · NIA · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI AGUILAR CALVO, PATRICIA · 2022 to 2024
$747kProbing prion clearance through interstitial fluid and perivascular pathwaysR21NS110409 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI SIGURDSON, CHRISTINA · 2018 to 2019
$433kMolecular basis of spongiform encephalopathy in prion diseaseR21NS105498 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI SIGURDSON, CHRISTINA · 2018 to 2019
$432kNCATS NIH HHS UL1 TR001442NIAMS NIH HHS R01 AR064202NIA NIH HHS K99 AG061251NIA NIH HHS P30 AG062429NIA NIH HHS R00 AG061251NIA NIH HHS R01 AG031189NIA NIH HHS R01 AG062562NINDS NIH HHS R01 NS069566NINDS NIH HHS R01 NS076896NINDS NIH HHS R01 NS103848NINDS NIH HHS R21 NS105498NINDS NIH HHS R21 NS110409NINDS NIH HHS R37 NS076896
Abstract
Cofactors are essential for driving recombinant prion protein into pathogenic conformers. Polyanions promote prion aggregation in vitro, yet the cofactors that modulate prion assembly in vivo remain largely unknown. Here we report that the endogenous glycosaminoglycan, heparan sulfate (HS), impacts prion propagation kinetics and deposition sites in the brain. Exostosin-1 haploinsufficient (Ext1
Indexed as
ADAM10 ProteinAnimalsBrainHeparan SulfateHumansMicePrion DiseasesPrionsADAM10 ProteinHeparan SulfatePrionsADAM10 cleavageAmyloidGlycosaminoglycansNeurodegeneration
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