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).

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 from infected individuals, usually by members of the same species.  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. These symptoms are due to lesions in the brain tissue.

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 fully accepted 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.

Viroids

Figure 1. These potatoes have been infected by the potato spindle tuber viroid. (credit: Pamela Roberts, University of Florida Institute of Food and Agricultural Sciences, USDA ARS)

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 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. For example, the potato spindle tuber viroid (PSTVd), which typically spreads when infected knives cut healthy potatoes in preparation for planting, can affect potatoes and tomatoes. The symptoms of PSTVd can be seen in Figure 1.