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By the end of this section, you will be able to:
  • Describe prions and their basic properties
  • Define viroids and their targets of infection

Prions and viroids are pathogens (agents with the ability to cause disease) that have simpler structures than viruses but, in the case of prions, still can produce deadly diseases.

Prions

Prions , so-called because they are proteinaceous, are infectious particles—smaller than viruses—that contain no nucleic acids (neither DNA nor RNA). Historically, the idea of an infectious agent that did not use nucleic acids was considered impossible, but pioneering work by Nobel Prize-winning biologist Stanley Prusiner has convinced the majority of biologists that such agents do indeed exist.

Fatal neurodegenerative diseases, such as kuru in humans and bovine spongiform encephalopathy (BSE) in cattle (commonly known as “mad cow disease”) were shown to be transmitted by prions. The disease was spread by the consumption of meat, nervous tissue, or internal organs between members of the same species. Kuru, native to humans in Papua New Guinea, was spread from human to human via ritualistic cannibalism. BSE, originally detected in the United Kingdom, was spread between cattle by the practice of including cattle nervous tissue in feed for other cattle. Individuals with kuru and BSE show symptoms of loss of motor control and unusual behaviors, such as uncontrolled bursts of laughter with kuru, followed by death. Kuru was controlled by inducing the population to abandon its ritualistic cannibalism.

On the other hand, BSE was initially thought to only affect cattle. Cattle dying of the disease were shown to have developed lesions or “holes” in the brain, causing the brain tissue to resemble a sponge. Later on in the outbreak, however, it was shown that a similar encephalopathy in humans known as variant Creutzfeldt-Jakob disease (CJD) could be acquired from eating beef from animals with BSE, sparking bans by various countries on the importation of British beef and causing considerable economic damage to the British beef industry ( [link] ). BSE still exists in various areas, and although a rare disease, individuals that acquire CJD are difficult to treat. The disease can be spread from human to human by blood, so many countries have banned blood donation from regions associated with BSE.

The cause of spongiform encephalopathies, such as kuru and BSE, is an infectious structural variant of a normal cellular protein called PrP (prion protein). It is this variant that constitutes the prion particle. PrP exists in two forms, PrP c    , the normal form of the protein, and PrP sc    , the infectious form. Once introduced into the body, the PrP sc contained within the prion binds to PrP c and converts it to PrP sc . This leads to an exponential increase of the PrP sc protein, which aggregates. PrP sc is folded abnormally, and the resulting conformation (shape) is directly responsible for the lesions seen in the brains of infected cattle. Thus, although not without some detractors among scientists, the prion seems likely to be an entirely new form of infectious agent, the first one found whose transmission is not reliant upon genes made of DNA or RNA.

Part a illustrates how normal prion protein (PRP) is converted into the disease-causing form (PRP). PRPsc may spontaneously form in brain tissue, may be introduced when a mutant form of the protein misfolds, or may introduced into the brain tissue by inoculation. The misfolded protein causes normal PRP already present in the brain to misfold. A chain reaction occurs, leading to a large amount of misfolded protein.
(a) Endogenous normal prion protein (PrP c ) is converted into the disease-causing form (PrP sc ) when it encounters this variant form of the protein. PrP sc may arise spontaneously in brain tissue, especially if a mutant form of the protein is present, or it may occur via the spread of misfolded prions consumed in food into brain tissue. (b) This prion-infected brain tissue, visualized using light microscopy, shows the vacuoles that give it a spongy texture, typical of transmissible spongiform encephalopathies. (credit b: modification of work by Dr. Al Jenny, USDA APHIS; scale-bar data from Matt Russell)

Viroids

Viroids are plant pathogens: small, single-stranded, circular RNA particles that are much simpler than a virus. They do not have a capsid or outer envelope, but like viruses can reproduce only within a host cell. Viroids do not, however, manufacture any proteins, and they only produce a single, specific RNA molecule. Human diseases caused by viroids have yet to be identified.

Viroids are known to infect plants ( [link] ) and are responsible for crop failures and the loss of millions of dollars in agricultural revenue each year. Some of the plants they infect include potatoes, cucumbers, tomatoes, chrysanthemums, avocados, and coconut palms.

The photo shows shriveled, cracked potatoes.
These potatoes have been infected by the potato spindle tuber viroid (PSTV), which is typically spread when infected knives are used to cut healthy potatoes, which are then planted. (credit: Pamela Roberts, University of Florida Institute of Food and Agricultural Sciences, USDA ARS)

Career connection

Virologist

Virology is the study of viruses, and a virologist is an individual trained in this discipline. Training in virology can lead to many different career paths. Virologists are actively involved in academic research and teaching in colleges and medical schools. Some virologists treat patients or are involved in the generation and production of vaccines. They might participate in epidemiologic studies ( [link] ) or become science writers, to name just a few possible careers.

This photo shows a scientist in a field, measuring the length of an egg.
This virologist is engaged in fieldwork, sampling eggs from this nest for avian influenza. (credit: Don Becker, USGS EROS, U.S. Fish and Wildlife Service)

If you think you may be interested in a career in virology, find a mentor in the field. Many large medical centers have departments of virology, and smaller hospitals usually have virology labs within their microbiology departments. Volunteer in a virology lab for a semester or work in one over the summer. Discussing the profession and getting a first-hand look at the work will help you decide whether a career in virology is right for you. The American Society of Virology’s website is a good resource for information regarding training and careers in virology.

Section summary

Prions are infectious agents that consist of protein, but no DNA or RNA, and seem to produce their deadly effects by duplicating their shapes and accumulating in tissues. They are thought to contribute to several progressive brain disorders, including mad cow disease and Creutzfeldt-Jakob disease. Viroids are single-stranded RNA pathogens that infect plants. Their presence can have a severe impact on the agriculture industry.

Questions & Answers

water as a function in cell for inorganic substances
chisom Reply
hi john....wat about for mineral salts, its function in a cell and structure
chisom
hi chisom which topic is this
Ezeh
this biology is for which grade?
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A1
Boadi
state any one specialized animal cell and it's function
Pascal Reply
sperm cell: Fertilization
Tshering
Can DNA and RNA be praticalized
Oyewale Reply
you can make models of them and show how they replicate
Oswald
what is dentition
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Study of teeths and their functions
Stanisla
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Tisa
it's not teeths. teeth is plural as well as singular noun. the function of the teeth is to cut and grind food for easier swallowing and for increasing the surface area if the fish as the substrate in which digestive enzymes work
Oswald
dentition is not the study of teeth but the arrangement of teeth in the mouth of species or individuals
Oswald
what are the two types of teeth
Tisa
Function of probosis
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why do we study biology?
SYDNEY Reply
where biology began
Alhaji
What are chromesome
Talkmore Reply
condensed DNA wrapped with histone
Elias
chromosomes are gins that we take from our parents.when mom get pregnant the baby inside is made up of 23 chromosomes from his dad and 23 chromosomes from his mom.so a baby is made up of totally 46 chromosomes
ezera
chromosome are rod or thread shape body located in the nucleus of a cell.
Alhaji
what is chromosome?
Royd Reply
How can a person who has been in the vascular disease repairs his or her DNA
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Shaf Reply
Of wat
John
explanation for wat
Christian
Of war na so dat I can provide
John
Is d process which food is converted into substance dat can be utilize by d body
John
the meaning of disgestion
Charity Reply
because they are considered to be proteins ..lipids,fats, and nucleic acids
Geeta Reply
what is osmosis
Festus Reply
Is the movement of solvent molecules from the region of a higher concentration to the region of lower concentration
Muhammad
It is the movement of solvent from the region of lower concentration to the region of higher concentration through a semi-permeable membrane.
Helen
and diffusion
Prudent
it is the movement of water molecules fro region of higher conc. to the region of lower conc.thro a semi-permeable membrane.
Chansa
Diffusion is the movement of solute molecules from a region of higher concentration to a region of lower concentration.
Quartey
osmosis is the movement of water molecule from the region higher water potential to a region of lower water potential
Pascal
what is the function of Nerve cell?
Pascal
Hello Everyone!! Any good study app recommendations?
HONNEA Reply
For which subject
Sinyinza
Biology:)
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Thanks!
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Source:  OpenStax, Biology. OpenStax CNX. Feb 29, 2016 Download for free at http://cnx.org/content/col11448/1.10
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