Aguilar-Calvo P, Sevillano AM, Bapat J, Soldau K, Sandoval DR, Altmeppen HC et al (2020) Shortening heparan sulfate chains prolongs survival and reduces parenchymal plaques in prion disease caused by mobile, ADAM10-cleaved prions. Acta Neuropathol 139:527–546. https://doi.org/10.1007/s00401-019-02085-x

Article  CAS  PubMed  Google Scholar 

Aguzzi A, Baumann F, Bremer J (2008) The prion’s elusive reason for being. Annu Rev Neurosci 31:439–477. https://doi.org/10.1146/annurev.neuro.31.060407.125620

Article  CAS  PubMed  Google Scholar 

Aguzzi A, Calella AM (2009) Prions: protein aggregation and infectious diseases. Physiol Rev 89:1105–1152. https://doi.org/10.1152/physrev.00006.2009

Article  CAS  PubMed  Google Scholar 

Altmeppen HC, Prox J, Krasemann S, Puig B, Kruszewski K, Dohler F et al (2015) The sheddase ADAM10 is a potent modulator of prion disease. Elife. https://doi.org/10.7554/eLife.04260

Article  PubMed  PubMed Central  Google Scholar 

Altmeppen HC, Prox J, Puig B, Kluth MA, Bernreuther C, Thurm D et al (2011) Lack of a-disintegrin-and-metalloproteinase ADAM10 leads to intracellular accumulation and loss of shedding of the cellular prion protein in vivo. Mol Neurodegener 6:36. https://doi.org/10.1186/1750-1326-6-36

Article  CAS  PubMed  PubMed Central  Google Scholar 

Altmeppen HC, Puig B, Dohler F, Thurm DK, Falker C, Krasemann S et al (2012) Proteolytic processing of the prion protein in health and disease. Am J Neurodegener Dis 1:15–31

PubMed  PubMed Central  Google Scholar 

Amin L, Nguyen XT, Rolle IG, D’Este E, Giachin G, Tran TH et al (2016) Characterization of prion protein function by focal neurite stimulation. J Cell Sci 129:3878–3891. https://doi.org/10.1242/jcs.183137

Article  CAS  PubMed  Google Scholar 

Ballmer BA, Moos R, Liberali P, Pelkmans L, Hornemann S, Aguzzi A (2017) Modifiers of prion protein biogenesis and recycling identified by a highly parallel endocytosis kinetics assay. J Biol Chem 292:8356–8368. https://doi.org/10.1074/jbc.M116.773283

Article  CAS  PubMed  PubMed Central  Google Scholar 

Beland M, Roucou X (2012) The prion protein unstructured N-terminal region is a broad-spectrum molecular sensor with diverse and contrasting potential functions. J Neurochem 120:853–868. https://doi.org/10.1111/j.1471-4159.2011.07613.x

Article  CAS  PubMed  Google Scholar 

Benilova I, Reilly M, Terry C, Wenborn A, Schmidt C, Marinho AT et al (2020) Highly infectious prions are not directly neurotoxic. Proc Natl Acad Sci USA 117:23815–23822. https://doi.org/10.1073/pnas.2007406117

Article  CAS  PubMed  PubMed Central  Google Scholar 

Bessen RA, Shearin H, Martinka S, Boharski R, Lowe D, Wilham JM et al (2010) Prion shedding from olfactory neurons into nasal secretions. PLoS Pathog 6:e1000837. https://doi.org/10.1371/journal.ppat.1000837

Article  CAS  PubMed  PubMed Central  Google Scholar 

Bieschke J, Weber P, Sarafoff N, Beekes M, Giese A, Kretzschmar H (2004) Autocatalytic self-propagation of misfolded prion protein. Proc Natl Acad Sci U S A 101:12207–12211. https://doi.org/10.1073/pnas.0404650101

Article  CAS  PubMed  PubMed Central  Google Scholar 

Boon BDC, Bulk M, Jonker AJ, Morrema THJ, van den Berg E, Popovic M et al (2020) The coarse-grained plaque: a divergent Abeta plaque-type in early-onset Alzheimer’s disease. Acta Neuropathol 140:811–830. https://doi.org/10.1007/s00401-020-02198-8

Article  CAS  PubMed  PubMed Central  Google Scholar 

Borchelt DR, Rogers M, Stahl N, Telling G, Prusiner SB (1993) Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor. Glycobiology 3:319–329

Article  CAS  PubMed  Google Scholar 

Borchelt DR, Scott M, Taraboulos A, Stahl N, Prusiner SB (1990) Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. J Cell Biol 110:743–752

Article  CAS  PubMed  Google Scholar 

Brenna S, Altmeppen HC, Mohammadi B, Rissiek B, Schlink F, Ludewig P et al (2020) Characterization of brain-derived extracellular vesicles reveals changes in cellular origin after stroke and enrichment of the prion protein with a potential role in cellular uptake. J Extracell Vesicles 9:1809065. https://doi.org/10.1080/20013078.2020.1809065

Article  CAS  PubMed  PubMed Central  Google Scholar 

Brummer T, Muller SA, Pan-Montojo F, Yoshida F, Fellgiebel A, Tomita T et al (2019) NrCAM is a marker for substrate-selective activation of ADAM10 in Alzheimer’s disease. EMBO Mol Med. https://doi.org/10.15252/emmm.201809695

Article  PubMed  PubMed Central  Google Scholar 

Calella AM, Farinelli M, Nuvolone M, Mirante O, Moos R, Falsig J et al (2010) Prion protein and Abeta-related synaptic toxicity impairment. EMBO Mol Med 2:306–314. https://doi.org/10.1002/emmm.201000082

Article  CAS  PubMed  PubMed Central  Google Scholar 

Castle AR, Kang SG, Eskandari-Sedighi G, Wohlgemuth S, Nguyen MA, Drucker DJ et al (2023) Beta-endoproteolysis of the cellular prion protein by dipeptidyl peptidase-4 and fibroblast activation protein. Proc Natl Acad Sci USA 120:e2209815120. https://doi.org/10.1073/pnas.2209815120

Article  CAS  PubMed  Google Scholar 

Chesebro B, Trifilo M, Race R, Meade-White K, Teng C, LaCasse R et al (2005) Anchorless prion protein results in infectious amyloid disease without clinical scrapie. Science 308:1435–1439. https://doi.org/10.1126/science.1110837

Article  CAS  PubMed  Google Scholar 

Collinge J (2001) Prion diseases of humans and animals: their causes and molecular basis. Annu Rev Neurosci 24:519–550

Article  CAS  PubMed  Google Scholar 

Corbett GT, Wang Z, Hong W, Colom-Cadena M, Rose J, Liao M et al (2020) PrP is a central player in toxicity mediated by soluble aggregates of neurodegeneration-causing proteins. Acta Neuropathol 139:503–526. https://doi.org/10.1007/s00401-019-02114-9

Article  CAS  PubMed  Google Scholar 

Curin Serbec V, Bresjanac M, Popovic M, Pretnar Hartman K, Galvani V, Rupreht R et al (2004) Monoclonal antibody against a peptide of human prion protein discriminates between Creutzfeldt–Jacob’s disease-affected and normal brain tissue. J Biol Chem 279:3694–3698. https://doi.org/10.1074/jbc.M310868200

Article  CAS  PubMed  Google Scholar 

Dexter E, Kong Q (2021) Neuroprotective effect and potential of cellular prion protein and its cleavage products for treatment of neurodegenerative disorders part II: strategies for therapeutics development. Expert Rev Neurother 21:983–991. https://doi.org/10.1080/14737175.2021.1965882

Article  CAS  PubMed  PubMed Central  Google Scholar 

Dohler F, Sepulveda-Falla D, Krasemann S, Altmeppen H, Schluter H, Hildebrand D et al (2014) High molecular mass assemblies of amyloid-beta oligomers bind prion protein in patients with Alzheimer’s disease. Brain 137:873–886. https://doi.org/10.1093/brain/awt375

Article  PubMed  Google Scholar 

Dorey A, Tholance Y, Vighetto A, Perret-Liaudet A, Lachman I, Krolak-Salmon P et al (2015) Association of cerebrospinal fluid prion protein levels and the distinction between Alzheimer disease and Creutzfeldt–Jakob disease. JAMA Neurol 72:267–275. https://doi.org/10.1001/jamaneurol.2014.4068

Article  PubMed  Google Scholar 

Dvorakova E, Vranac T, Janouskova O, Cernilec M, Koren S, Lukan A et al (2013) Detection of the GPI-anchorless prion protein fragment PrP226* in human brain. BMC Neurol 13:126. https://doi.org/10.1186/1471-2377-13-126