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    Home > Active Ingredient News > Study of Nervous System > The prion disease that must die after the onset of disease ushered in a turnaround, and its lethal mechanism was cracked

    The prion disease that must die after the onset of disease ushered in a turnaround, and its lethal mechanism was cracked

    • Last Update: 2022-01-10
    • Source: Internet
    • Author: User
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    Written | Edited by Wang Cong | Typeset by Wang Duoyu | Prion, strictly speaking, is not a virus.
    They do not contain nucleic acid but are composed only of protein.
    They are a class of self-replicating and infectious small molecules that are hydrophobic Protein can cause central nervous system diseases in mammals and humans


    Human prion disease is an incurable and fatal neurodegenerative disease, usually manifested as rapidly progressive dementia

    Regardless of whether the cause is sporadic, hereditary or acquired, and whether the clinical name is Creutzfeldt-Jakob disease, fatal familial insomnia, or GSS syndrome, all prion diseases are caused by a single molecular mechanism

    That is, it is caused by the change in the conformation of the prion protein (PrP) encoded by the PRNP gene from its inherently folded form (PrPC) to a self-replicating misfolded form (PrPSc)

    The research on prions has won two Nobel Prizes in Physiology or Medicine.
    We have already figured out the pathogenicity and transmission mechanism of prions.
    However, why prions cause brain neuron death is still not fully understood


    We have reported two very representative patients with prion diseases.
    They represent two types of prion diseases


    One is Émilie Jaumain.
    At the age of 24 in 2010, she was doing brain slices of a mouse model of sheep-adaptive mad cow disease in the laboratory.
    She stabbed her finger outside and was infected with prions


    Nine years later, she died of Creutzfeldt-Jakob disease caused by prion infection

    The other is Sonia Vallabh.
    She inherited a mutation in the PRNP (D178N) gene from her mother.
    This gene mutation will cause a type of prion disease-fatal familial insomnia.
    The disease usually develops at the age of 50 and occurs very quickly.
    Caused death, she is currently 37 years old


    On December 22, 2021, researchers from the Scripps Institute in the United States published a research paper titled Endosomal sorting drives the formation of axonal prion protein endoggresomes in the Science Advances journal, which was also selected as the cover paper of the current issue

    The study found that protein aggregates formed by prion proteins can enter and destroy the axons of neurons, thereby killing neurons, leading to the gradual loss of brain function, and ultimately death

    Other neurodegenerative diseases (such as Alzheimer’s disease and Parkinson’s disease) also have signs of accumulation of protein aggregates and swelling of neuronal axons in the early stages.
    Therefore, the significance of this discovery may be far more significant than prion diseases and may be useful The more common neurodegenerative diseases are of great significance


    Normally, neurons can remove harmful proteins around their cell bodies, but they can't do anything about the axon area

    The research team discovered 4 kinds of proteins-Arl8, kinesin-1, Vps41 and SKIP, they are involved in the packaging and transport of prion proteins into the axons of neurons, and promote the formation of aggregates of prion proteins

    The research team further discovered that by inhibiting any of these four proteins, the prion protein cannot enter the neuron axons

    The discovery that prion protein (cyan) is enriched in human neuron axons may be far more significant than prion diseases, and may have important implications for more common neurodegenerative diseases

    It suggests that we may be able to treat a variety of neurodegenerative diseases by preventing harmful proteins from entering neurons

    The discovery of prions In 1957, American scientist Daniel Gajdusek discovered the Fore tribe in the eastern highlands of Papua New Guinea.
    Many people of the Fore tribe suffered from a strange disease called Kuru disease.
    , The incubation period is long, the patients in the early stage only feel headache and joint pain, followed by ataxia, tremor, and involuntary movement.
    The latter includes chorea, myoclonus, etc.
    , and progressively worsening dementia and neurological abnormalities occur in the late course of the disease.


    Most patients die within 3 to 6 months of onset

    At first, Gedusek thought that Kuru’s disease was a genetic disease.
    Later, he discovered that the Fore people had the custom of eating the organs of their deceased relatives as a nostalgia.
    After research, Gedusek found that Kuru’s disease is a kind of gastrointestinal disease.
    The spread of infectious diseases of unknown causative agent


    Under Geduzek’s persuasion, the Fore people abolished this custom, Kuru disease has since disappeared, and no new cases have been found

    Gedusek also won the 1976 Nobel Prize in Physiology or Medicine for discovering Kuru's disease, a new type of pathogenicity and transmission mechanism, and eliminating this deadly disease

    The source of Kuru's disease is the later famous Prion (Prion)

    Creutzfeldt-Jakob disease is a rare transmissible brain disease that mainly occurs between the ages of 50 and 70.
    Infected people can have sleep disturbances, personality changes, ataxia, aphasia, vision loss, physical, and muscle atrophy.
    , Myoclonus, progressive dementia and other symptoms, and will die within one year of onset


    The disease was discovered as early as 1922, but the cause of the disease has not been known and cannot be cured

    Creutzfeldt-Jakob disease is the most common prion disease in humans.
    The incidence rate is very low, less than one in a million.
    At present, more than 6000 people get sick every year in the world.


    In 1982, the American biochemist Stanley Prusiner (Stanley Prusiner) published a paper in Science and successfully isolated the causative agent of Creutzfeldt-Jakob disease and named it Prion

    In 1991, Stanley Bruchner published a paper in Science, revealing the pathogenic mechanism of prions, thereby elucidating the causes of animal scrapie and human Creutzfeldt-Jakob disease

    He also won the Nobel Prize in Physiology or Medicine in 1997

    The link to the paper: https:// is open for reprinting, welcome to forward to Moments and WeChat groups 
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