Cited by 455Prion Diseases and Protein MisfoldingAlzheimer's disease research and treatmentsAmyotrophic Lateral Sclerosis Research

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Biology and Genetics of Prions Causing Neurodegeneration

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Prusiner, Stanley B.·2013·Annual Review of Genetics

DOI 10.1146/annurev-genet-110711-155524PMC PMC4010318

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Abstract

Prions are proteins that acquire alternative conformations that become self-propagating. Transformation of proteins into prions is generally accompanied by an increase in β-sheet structure and a propensity to aggregate into oligomers. Some prions are beneficial and perform cellular functions, whereas others cause neurodegeneration. In mammals, more than a dozen proteins that become prions have been identified, and a similar number has been found in fungi. In both mammals and fungi, variations in the prion conformation encipher the biological properties of distinct prion strains. Increasing evidence argues that prions cause many neurodegenerative diseases (NDs), including Alzheimer's, Parkinson's, Creutzfeldt-Jakob, and Lou Gehrig's diseases, as well as the tauopathies. The majority of NDs are sporadic, and 10% to 20% are inherited. The late onset of heritable NDs, like their sporadic counterparts, may reflect the stochastic nature of prion formation; the pathogenesis of such illnesses seems to require prion accumulation to exceed some critical threshold before neurological dysfunction manifests.

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See all 455

Authors: Prusiner, S. B.
Affiliations: University of California, San Francisco (US)
Corresponding author: Prusiner, Stanley B.
ORCID: Prusiner, Stanley B.
Venue: Annual Review of Genetics
Publisher: Annual Reviews
Published: 2013-11-23
Volume: 47
Issue: 1
Pages: 601-623
ISSN: 0066-4197 (ISSN-L)
1545-2948
DOI: 10.1146/annurev-genet-110711-155524
PMC: PMC4010318
OpenAlex: W2150555206
Cited by: 455
References: 207
Citations by year: 2026: 12 · 2025: 18 · 2024: 30 · 2023: 22 · 2022: 27 · 2021: 27 · 2020: 36 · 2019: 45 · 2018: 47 · 2017: 55 · 2016: 61 · 2015: 45 · 2014: 28
FWCI: 4.20
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Locations: Annual Review of Genetics (publishedVersion)
PubMed (publishedVersion)
Europe PMC (PubMed Central) (acceptedVersion)
PubMed Central (acceptedVersion)
eScholarship (California Digital Library) (submittedVersion)
Funders: National Institutes of Health
National Institute on Aging (AG010770, P01AG002132, P01AG021601, P01AG010770, AG002132, AG 021601, AG031220, R37AG031220)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords: prions, prion strains, protein conformation, protein aggregation, neurodegenerative diseases, Alzheimer's disease, Parkinson's disease, Creutzfeldt-Jakob disease, amyotrophic lateral sclerosis, tauopathy, hereditary neurodegeneration, prion pathogenesis, fungi
MeSH terms: Animals, Inclusion Bodies, Fungal Proteins, Humans, Mammals, Models, Molecular, Peptide Termination Factors, Prions, Protein Conformation, Transcription Factors, Virulence, Neurofibrillary Tangles, tau Proteins, Prion Diseases, Age of Onset, Neurodegenerative Diseases, Tauopathies, Saccharomyces cerevisiae Proteins, mRNA Cleavage and Polyadenylation Factors, Synucleins, Plaque, Amyloid, Amyloidogenic Proteins

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Biology and Genetics of Prions Causing Neurodegeneration

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