Frank O. Bastian is an American

medical doctor A physician (American English), medical practitioner (Commonwealth English), medical doctor, or simply doctor, is a health professional who practices medicine, which is concerned with promoting, maintaining or restoring health through th ...

and

neuropathologist Neuropathology is the study of disease of nervous system tissue, usually in the form of either small surgical biopsies or whole-body autopsies. Neuropathologists usually work in a department of anatomic pathology, but work closely with the clinic ...

, who previously worked at

Louisiana State University Louisiana State University (officially Louisiana State University and Agricultural and Mechanical College, commonly referred to as LSU) is a public land-grant research university in Baton Rouge, Louisiana. The university was founded in 1860 nea ...

, moved to a university in New Orleans in 2019. He specializes in the

transmissible spongiform encephalopathies Transmissible spongiform encephalopathies (TSEs) are a group of progressive and fatal conditions that are associated with prions and affect the brain and nervous system of many animals, including humans, cattle, and sheep. According to the most ...

(TSEs), which include, but are not limited to,

Bovine spongiform encephalopathy Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is an incurable and invariably fatal neurodegenerative disease of cattle. Symptoms include abnormal behavior, trouble walking, and weight loss. Later in the course of t ...

(BSE) "Mad cow disease" in cattle,

scrapie Scrapie () is a fatal, degenerative disease affecting the nervous systems of sheep and goats. It is one of several transmissible spongiform encephalopathies (TSEs), and as such it is thought to be caused by a prion. Scrapie has been known since ...

in sheep and goats, and

Creutzfeldt–Jakob disease Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is an invariably fatal degenerative brain disorder. Early symptoms include memory problems, behavioral changes, ...

(CJD) in humans. Bastian challenges the widely popular theory that the TSEs are caused by

prions Prions are misfolded proteins that have the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several fatal and transmissible neurodegenerative diseases in humans and many other animals. It i ...

, which are composed mostly if not entirely of a misfolded form of a protein called PrP (i.e. PrP^Sc). He claims instead that the TSEs are caused by an extremely small,

cell wall A cell wall is a structural layer surrounding some types of cells, just outside the cell membrane. It can be tough, flexible, and sometimes rigid. It provides the cell with both structural support and protection, and also acts as a filtering mech ...

deficient

bacterium Bacteria (; singular: bacterium) are ubiquitous, mostly free-living organisms often consisting of one biological cell. They constitute a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria were among ...

called ''

Spiroplasma ''Spiroplasma'' is a genus of Mollicutes, a group of small bacteria without cell walls. ''Spiroplasma'' shares the simple metabolism, parasitic lifestyle, fried-egg colony morphology and small genome of other ''Mollicutes'', but has a distinctive ...

'', and that prions are the result of the disease process. The lack of a cell wall means it is not susceptible to most common

antibiotic An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting bacterial infections, and antibiotic medications are widely used in the treatment and prevention of ...

s such as

penicillin Penicillins (P, PCN or PEN) are a group of β-lactam antibiotics originally obtained from ''Penicillium'' moulds, principally '' P. chrysogenum'' and '' P. rubens''. Most penicillins in clinical use are synthesised by P. chrysogenum using ...

, which target cell wall synthesis. Bastian first found ''Spiroplasma'' in the brain of a CJD patient in an

autopsy An autopsy (post-mortem examination, obduction, necropsy, or autopsia cadaverum) is a surgical procedure that consists of a thorough examination of a corpse by dissection to determine the cause, mode, and manner of death or to evaluate any di ...

in 1979. He later demonstrated that '' Spiroplasma mirum'' strain SMCA (suckling mouse cataract agent) induces a microcystic spongiform encephalopathy in suckling rats that is similar to rat CJD. In 2007 he isolated ''Spiroplasma'' from scrapie and

Chronic wasting disease Chronic wasting disease (CWD), sometimes called zombie deer disease, is a transmissible spongiform encephalopathy (TSE) affecting deer. TSEs are a family of diseases thought to be caused by misfolded proteins called prions and include similar dis ...

(a TSE affecting

deer Deer or true deer are hoofed ruminant mammals forming the family Cervidae. The two main groups of deer are the Cervinae, including the muntjac, the elk (wapiti), the red deer, and the fallow deer; and the Capreolinae, including the reindeer ...

elk The elk (''Cervus canadensis''), also known as the wapiti, is one of the largest species within the deer family, Cervidae, and one of the largest terrestrial mammals in its native range of North America and Central and East Asia. The common ...

, and

moose The moose (in North America) or elk (in Eurasia) (''Alces alces'') is a member of the New World deer subfamily and is the only species in the genus ''Alces''. It is the largest and heaviest extant species in the deer family. Most adult mal ...

, abbreviated CWD), cultured them, and inoculated them into sheep and deer. According to Bastian, the scrapie and CWD ''Spiroplasma'' isolates caused a spongiform encephalopathy identical to scrapie and CWD. Under his hypothesis, because PrP^Sc is not the cause it is merely an imperfect marker of infection (with both sensitivity and NPV <1), and is either induced by ''Spiroplasma'' directly or by a defence mechanism of the host. In 2014 he discovered that ''Spiroplasma'' forms

biofilms A biofilm comprises any syntrophic consortium of microorganisms in which cells stick to each other and often also to a surface. These adherent cells become embedded within a slimy extracellular matrix that is composed of extracellular ...

which give them significant resistance to radiation, heat, and disinfectants. He, along with

Laura Manuelidis Laura Manuelidis is a physician and neuropathologist at Yale University. Career Manuelidis earned her B.A. degree from Sarah Lawrence College, where she studied poetry, and her M.D. is from Yale Medical School. She is head of the section of Neur ...

Yale University Yale University is a private research university in New Haven, Connecticut. Established in 1701 as the Collegiate School, it is the third-oldest institution of higher education in the United States and among the most prestigious in the wo ...

, who claims that the TSEs are caused by an as yet undiscovered

virus A virus is a submicroscopic infectious agent that replicates only inside the living cells of an organism. Viruses infect all life forms, from animals and plants to microorganisms, including bacteria and archaea. Since Dmitri Ivanovsky's 1 ...

, are two of the main sceptics of the

prion Prions are misfolded proteins that have the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several fatal and transmissible neurodegenerative diseases in humans and many other animals. It ...

theory. Other scientists have so far been unable to duplicate his results, casting doubt on this hypothesis. Bastian however maintains that the inability to detect Spiroplasma in 100% of cases stems from genetic variability of cases.

Publications

References

{{DEFAULTSORT:Bastian, Frank O. Living people American neurologists American pathologists Prions Year of birth missing (living people)

Publications

See also

References