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Table 1 Prion proteins: bona fide prions, prion candidates, and prionoids

From: Protein assembly systems in natural and synthetic biology

PrionProtein determinantFunctionPrion phenotypeOrganismPrion propertiesReference
Amyloid AApolipoprotein, inflammatory responseAmyloid A amyloidosisHuman, cattle, cheetah, chicken, mouseAmyloid, cell-to-cell spreading, trans-organismal spreading (except for humans)[43]
[β]Prb1Vacuolar proteinase B, protein degradation in vacuolePhenotypic lag: prolonged carboxypeptidase Y activity after loss of PEP4Saccharomyces cerevisiaeNon-amyloid, reversibly curable, mitotic inheritance, infectious[55]
CPEB, Orb2, CPEB3*Regulating synaptic plasticity, repressor of AMPA receptor transcriptionFacilitating long-term memory, activator of AMPA receptor translationSea slug, fruit fly, mouseSelf-templating, amyloid, heritable in yeast[56,57,58]
Cb-Rho prion*RhoTranscriptional global regulatorTranscriptional terminator read-throughClostridium botulinum, Escherichia coliAmyloid, Sup35C assay, inheritance[59]
[GAR+]Pma1/Std1Proton pump, glucose signalingUtilization of poor carbon sources in the presence of glucoseSaccharomyces cerevisiaeInheritance, infectious[54, 60]
[Het-s]Het-sHeterokaryon incompatibility upon mixing with selfPodospora anserinaDependence on Pa Hsp104, amyloid fibrils infectious[61, 62]
[LD+]*Lumini-dependensTranscription factor controlling autonomous flowering pathwayHypothetical: delay of floweringArabidopsis thalianaSup35C assay, detergent resistant aggregates, Hsp70 and Hsp90 dependence, inheritance, infectious[63, 64]
[LSB+]Lsb2Cytoskeletal assembly proteinSeeds [PSI+]Saccharomyces cerevisiaeDependence on Hsp104, inheritance, infectious, detergent resistant aggregate[65]
[MOD+]Mod5tRNA isopentenyltransferaseResistance to azole antifungals, modified sterol biosynthesisSaccharomyces cerevisiaeAmyloid, Hsp104 dependence, inheritance, infectious[66, 67]
[MOT3]Mot3Transcriptional regulationIncreased biofilm formation, agar invasion, decreased hypoxia resistanceSaccharomyces cerevisiaeAmyloid, dependence on Hsp104, inheritance, infectious[60, 68]
[NSI+]UnknownUnknownTranslational read-through affecting the Sup45 termination factorSaccharomyces cerevisiaeSup35C assay, dependence on Hsp104, inheritance, infectious[60, 69]
[NUP100+]Nup100Nuclear pore complexNo significant effectSaccharomyces cerevisiaeAmyloid, self-assembly, Sup35C assay, dependence on Hsp104, mitotic inheritance[67, 70, 71]
[OCT+]Cyc8Ttranscriptional regulation, chromatin regulationDerepression of genes inhibited by Cyc8-Tup1 complex, invertase production in the presence of glucose, flocculationSaccharomyces cerevisiaeDependence on Hsp104, inheritance, infectious[60, 72]
[PSI+]Sup35Translational terminationTranslational read-throughSaccharomyces cerevisiaeAmyloid, Sup35C assay, dependence on Hsp104, inheritance, infectious[60, 67, 73, 74]
PrP ScPRNPPreserves synaptic structure and functionFatal neurodegenerative diseasesHuman, sheep, cattle, deer, mink, felinesSelf-templating, detergent and protease resistant amyloid fibers, infectious[75]
[RNQ+]Rnq1UnknownCross-seeding other prionsSaccharomyces cerevisiaeAmyloid, Sup35C assay, dependence on Hsp104, inheritance, infectious[60, 67, 73, 74]
[SWI+]Swi1Transcriptional regulation, nucleosome remodelingPoor growth on non-glucose carbon sources, abolished multicellular featuresSaccharomyces cerevisiaeAmyloid, Sup35C assay, dependence on Hsp104, inheritance, infectious[60, 67, 73, 74]
[URE3]Ure2Gln3 repressorUtilization of poor nitrogen sources in the presence of ammoniumSaccharomyces cerevisiaeAmyloid, Sup35C assay, inheritance, infectious[60, 67, 73, 74]
PrionoidProtein determinantFunctionPrion phenotypeOrganismPrionoid propertiesReference
ASCAdaptor protein for inflammasome signalingInflammasome activationHumanNon-amyloid helical polymer, cell-to-cell spreading
PYD domain in yeast: Sup35C assay, detergent resistant aggregates, inheritance
[76, 77]
[LEF+]LEF-10Viral late expression factorDownregulation of viral late gene expressionAutographa californica multiple nucleopolyhedrovirusSup35C assay, detergent resistant aggregates[78]
MAVS*Signal transduction from mitochondrial membrane to cytosolActivation of MAVS, initiation of antiviral signalingHuman, mouseSelf-templating, detergent and protease-resistant fibers[79]
[NU+]New1 PrDATP binding cassette proteinSusceptibility to [PSI+] inductionSaccharomyces cerevisiaeSup35C assay, amyloid,
full New1 protein not shown to form prions
[74, 80] [68]
[NRP+]Nrp1**Putative RNA binding protein Saccharomyces cerevisiaeSup35C assay, detergent-resistant aggregates, Hsp104 dependence[68, 74]
p53Transcriptional regulatorMetastasisHuman, mouseLarge cytoplasmic inclusions, cell-to-cell spreading[43]
Islet amyloid polypeptide (IAPP)Glycemic regulationType 2 diabetesHuman, mouseCell-to-cell spreading, self-templating amyloid[43]
Prion candidatesOrganismReference
Amyloid A, amyloid-β, −synuclein, β2-microglobulin, immunoglobulin light chain, tau, transthyretinHuman[43]
ASM4, CBK1, GLN3, GPR1, GTS1, HRP1, KSP1, LSM4, NGR1, NRP1, NSP1, PDR1, PGD1, PUB1, PUF2, RBS1, RLM1, SAP30, YBL081W, YBR016W, YPL184C, YPR022CSaccharomyces cerevisiae[68]
ASH1, AZF1, BUD2, CSR2, ERG11, FRE1, GMC1, HAA1, HAP4, HEH2, HRD3, ILV1, JNM1, KAP120, KAP95, MGA1, MPH1, MRN1, MRPL10, MRS3, PBP2, PCL9, PIB1, POL32, PSP1, PUS4, RBS1, RLM1, SAP1, SBE2, SCD5, SED5, SEN15, SLI15, SMP1, SNT1, SPC110, STE20, STE5, UBX7, ULP1, VTS1, YCK3, YGL036W, YFH1, YLR152CSaccharomyces cerevisiae[81]
  1. *Validation in non-native host organism. **Incomplete validation. In the prion nomenclature, brackets denote non-Mendelian inheritance and capital letters denote dominance in crosses. Prion properties include: amyloid—prion was shown to form amyloid or detergent resistant aggregates; inheritance—prion is inherited mitotically, meiotically, or as a fusion protein (Sup35C assay); chaperone dependence—prion is dependent on particular chaperones for propagation. Prion candidates are proteins that cannot officially be classified as bona fide prions because they lack experimental validation, but have the potential for inter-organismal spreading