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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

there are 3 trimester in human pregnancy
ROHIN Reply
I don't know answer of this question can u help me
ROHIN
yes
Bisa
what is a cell
Fatima Reply
what is genetic
Janet Reply
I join
Janet
what are the branchas of biology
Prisca Reply
zoology, ecology
Millicent
biochemistry,cytology,herpetology...etc
R0se
genetics, microbiology,botany and embryology
Muhammad
what is a cell
Kulunbawi Reply
cell is smallest unit of life. cells are often cell the building blocks of life...
Muhammad
the first twenty element
Orapinega Reply
what are the characteristics of living things?
R0se
growth,respiration,nutrition,sensitivity, movement,irritability, excretion,death.
Obinna
What is the difference between adaptation and competition in animals
Adeyemi Reply
What is biology
Adeyemi
it is a natural science stadey about living things
Zamiil
Biology is the bronch of science which deals with the study of life is called biology
Aziz
what is the x in 300 stands for?
Ogbudu Reply
the properties of life
Clarinda Reply
response to the environment, reproduction, homeostasis, growth,energy processing etc.....
Pushpam
hello.
Daniela
hi
MacPeter
Good
Thomas
what is reproduction
Tims
Reproduction is a fundamental feature of all known life,each individual organism exist as a result of re production.....or else Multiplying...
R0se
a complete virus particle known as
Darlington Reply
These are formed from identical protein subunitscalled capsomeres.
Pushpam
fabace family plant name
Pushpam Reply
in eukaryotes ...protein channel name which transport protein ...
Pushpam Reply
in bacteria ...chromosomal dna duplicate structure called
Pushpam
what is a prokaryotic cell and a eukaryotic cell
Matilda Reply
There are two types of cells. Eukaryotic and Prokaryotic cells. Prokaryotic cells don't have a nucleus or membrane enclosed organelles (little organs within that cell). They do however carry genetic material but it's not maintained in the nucleus. Prokaryotic cells are also one celled.
juanita
Prokaryotic cells are one celled (single celled).
juanita
Prokaryotic cells are Bacteria and Archea
juanita
Prokaryotic cells are smaller than Eukaryotic cells.
juanita
Eukaryotic cells are more complex. They are much bigger than Prokaryotic cells.
juanita
Eukaryotic cells have a nucleus and membrane bound organelles.
juanita
Eukaryotic cells are animals cells which also includes us.
juanita
Eukaryotic cells are also multicellular.
juanita
nice explaination
Amna
eukaryotic cells are individual cells .. but eukaryotes are multicellular organisms which consist of many different types of eukaryotic cells
Will
also eukaryotic cells have mitochondria. prokaryotic cells do not
Will
Good
John
in prokaryotes only ribosomes are present... in eukaryotes mitochondria ...glogi bodies ..epidermis .....prokaryotes one envelop but eukaryotes compartment envelop....envelop mean membrane bound organelles......
Pushpam
prokaryotic cell are cells dat have no true nuclei i.e no cell membrane while eukaryotic cell are cell dat have true nuclei i.e have cell membrane
Divine
grt
Thomas
we have 46 pair of somatic cell and 23 pair of chromosomes in our body, pls can someone explain it to me. pls
Matilda Reply
we have 22 pairs of somatic chromosomes and one pair of sex chromosome
Amna
thanks
Matilda
we have 23 pairs of chromosomes,22 pairs of somatic and one pair of sex chromosomes
Amna
23 chromosomes from dad & 23 chromosomes from mom 23 +23=46 total chromosomes
juanita
X & Y chromosomes are called sex cells, the very presence of a Y chromosome means the person is Male.
juanita
XX Female XY Male
juanita
If a Karyotype has more than 46 Chromosomes then nondisjunction occured. For example, having an extra chromosome 21 will cause Down Syndrome.
juanita
in mammal state the different vertebrae and their location in the body
Igbinigie Reply

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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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