Prusiner prions. Medical search. Frequent questions

In 1997 Stanley Prusiner was awarded the Nobel Prize in physiology or medicine for a revolutionary theory about the mechanisms of infection. (jrank.org)
Stanley Prusiner won the 1997 Nobel prize in physiology or medicine for his research on prions. (jrank.org)
A year later, neurologist Dr. Stanley Prusiner was studying TSE diseases and discovered that a very small protein could become misshapen and resistant to the body's ability to take it apart. (theconversation.com)
Dr. Stanley Prusiner, the Nobel laureate who discovered prions, contradicted those claims and explained that he believes the disease is already widespread in the United States. (harpers.org)
Seitz mentioned the work of Stanley Prusiner, who won the Nobel prize for his research into prions (Prusiner even thanks Seitz and RJ Reynolds in his Nobel Prize acceptance speech which you can read here). (populartechnology.net)
In 1982 Stanley Prusiner was able to isolate a suspected infectious agent, a protein that he called a prion. (eyebulb.com)
Why Stanley Prusiner is famous? (eyebulb.com)
Stanley Prusiner won the Nobel Prize in Physiology or Medicine in 1997 for his work in proposing an explanation for the cause of bovine spongiform encephalopathy ("mad cow disease") and its human equivalent, Creutzfeldt-Jakob disease. (eyebulb.com)
Why did Stanley Prusiner win the Nobel Prize in Medicine or Physiology in 1997 be very specific and include a detailed answer that describes his work? (eyebulb.com)
The 1997 Nobel Prize in Physiology or Medicine is awarded to the American Stanley Prusiner for his pioneering discovery of an entirely new genre of disease-causing agents and the elucidation of the underlying principles of their mode of action. (eyebulb.com)
Who was Stanley Prusiner and what did he do? (eyebulb.com)
When did Stanley Prusiner win the Nobel Prize? (eyebulb.com)
When did Stanley Prusiner isolate the prion protein? (eyebulb.com)
Stanley Prusiner와 공동연구원들은 CWD에 감염된 뮬사슴의 변에서 증상이 나타나기 훨씬 전부터 감염성 프리온이 분비되기 시작한다는 사실을 발견하였다. (natureasia.com)

[ 5 ] Prusiner introduced the term prion to indicate that scrapie is related to a proteinaceous infectious particle (PrP). (medscape.com)
For example, while no one knows how or where CWD originated, some scholars think a mutated prion jumped the species barrier to deer from sheep infected with another animal prion disease called scrapie . (theconversation.com)
Prusiner showed that in lesions in animals with the neuro-degenerative disease scrapie, there is an abnormal form of this protein. (wolffund.org.il)
Err l a jellegzetes mozg sr l kapta a betegs g mind angol, mind magyar nev t (scrapie, surl k r). rdekes, hogy a hum n prion betegs gekkel ellent tben a surl kor a juhok s kecsk k k z tt kontakt ton is terjed (3). (oszk.hu)
A csontliszt k sz t s hez m r kor bban is felhaszn ltak juhbels s geket, amelyek k z tt scrapie-vel fert z tt llatok szervei is lehettek, azonban a technol giav lt st megel z en nem tudunk prion betegs g el fordul s r l a szarvasmarh k k z tt. (oszk.hu)
Scrapie is a contagious prion disease [ 1 ] of sheep and goat. (biomedcentral.com)
Susceptibility to scrapie is largely controlled by three polymorphic amino acid positions (136, 154, 171) of the ovine prion protein gene ( prnp ) [ 4 ] and reliable genotyping of the corresponding DNA polymorphism is required as a basis for selection decisions. (biomedcentral.com)
Prusiner discovered prions while investigating transmissible spongiform encephalopathies, including scrapie and Creutzfeldt-Jakob disease (CJD). (eyebulb.com)
Mammalian prion diseases include human Creutzfeldt-Jakob disease (CJD), bovine spongiform encephalopathy (BSE), sheep scrapie, and cervid chronic wasting disease (CWD). (biomedcentral.com)

These amyloid plaques are immunoreactive with antibodies to the prion protein and do not immunoreact with antibodies to other amyloidogenic proteins, such as the amyloid-beta (which is deposited in Alzheimer disease). (medscape.com)
Nevertheless, there is a strong correlation between the neurotoxicity caused by prion proteins and the blockade of their normal proteolysis. (mdpi.com)
It has been suggested that cell-to-cell spread requires that abnormal cytosolic proteins in ALS, for example, TDP-43, which seems to underlie the ubiquitinated inclusions so characteristic of sporadic ALS, are misfolded, self-aggregate and form toxic proteinaceous inclusions with a β cross-conformation, resembling prions. (bmj.com)
What distinguishes amyloid ﬁbrils formed by prions from those formed by other proteins is not clear. (pdf-archive.com)
Hence, what diﬀerentiates amyloids formed by bona fide prions from amyloids formed by other proteins is not well understood. (pdf-archive.com)
Recent studies indicate that nanomechanics may play an important role not only in the conversion process of soluble proteins into their ﬁbrillar state, but especially in the key characteristics of prions: their transmissibility.8,9 Amyloid ﬁbrils are highly sensitive to local thermal ﬂuctuations in liquid medium, which cause them to undergo bending along their longitudinal axis. (pdf-archive.com)
Mad Cow disease is caused by unconventional pathogens called prions--literally infectious proteins--which, because of their unique structure, are practically invulnerable, surviving even incineration[10] at temperatures hot enough to melt lead. (curezone.com)
According to Dr. Prusiner's findings, harmless forms of prions exist as proteins in the brain. (eyebulb.com)
For Stanley B. Prusiner, M.D., vindication came in 1997 when he won the Nobel Prize in Physiology or Medicine for his discovery of the potentially infectious proteins he called prions. (eyebulb.com)
During the 20 years leading up to the honor, Prusiner was seen as a heretic for his view that "rogue" proteins could cause disease. (eyebulb.com)
Written By: Stanley B. Prusiner, in full Stanley Ben Prusiner, (born May 28, 1942, Des Moines, Iowa, U.S.), American biochemist and neurologist whose discovery in 1982 of disease-causing proteins called prions won him the 1997 Nobel Prize for Physiology or Medicine. (eyebulb.com)
Proteins showing prion behaviour are also found in some fungi . (wikidoc.org)
However, the prion protein found in infectious material has a different structure and is resistant to proteases , the enzymes in the body that can normally break down proteins. (wikidoc.org)
Structural Studies of Prion Proteins. (ac.be)

Highly divergent hypotheses have been put forward regarding the makeup of the prions, including that they consist of nucleic acid only or protein only, are lacking both protein and nucleic acid, or are a polysaccharide. (medscape.com)
This hypothesis suggests that prions contain no nucleic acid and are referred to as PrP Sc . (medscape.com)
While the latter rely on nucleic acid for survival and replication, the prion is made of a protein and lacks nucleic acid. (jrank.org)
Before the work of Professor Stanley B. Prusiner, infectious diseases were regarded as exclusively caused by nucleic acid-containing agents such as viruses, bacteria and parasites. (wolffund.org.il)
Prusiner demonstrated that the agents responsible for their transmission, which are called prions, were composed only of protein and were devoid of nucleic acid. (wolffund.org.il)

The replication of prions involves the recruitment of the normally expressed prion protein, which has mainly an alpha-helical structure, into a disease-specific conformation that is rich in beta-sheet. (medscape.com)
Camacho MV, Telling G , Kong Q , Gambetti P , Notari S . Role of prion protein glycosylation in replication of human prions by protein misfolding cyclic amplification. (academictree.org)

Prion diseases, or transmissible spongiform encephalopathies, comprise a group of fatal neurodegenerative disorders in humans and animals for which there are no effective treatments or cures. (elifesciences.org)
These diseases are fatal neurologic diseases known as transmissible spongiform encephalopathies (TSEs), and they result from the misfolding of the normal cellular prion protein (PrP C ) into a pathogenic form (PrP Sc ) that accumulates primarily in the central nervous system [ 1 , 2 , 3 , 4 ]. (biomedcentral.com)
Prion related neurodegenerative diseases, called transmissible spongiform encephalopathies, are observed in many animal species. (ecmbio.com)

This hypothesis was initially greeted with great skepticism in the scientific community but eventually became widely accepted, allowing Prusiner to win the Noble Prize in Physiology or Medicine 1997. (medscape.com)
His theory, the "prion hypothesis," concerns an unusual protein, the prion, which occurs in the complete absence of DNA and RNA. (jrank.org)
While several critical issues remain to be addressed, the prion hypothesis may furnish a plausible framework to understand the pathogenesis of several deadly brain diseases of the central nervous system. (jrank.org)
The prion hypothesis received unexpected support from the simplest of eukaryotic organisms - yeast. (eyebulb.com)
What is the prion hypothesis? (eyebulb.com)
This is not surprising considering that the prion hypothesis claims that a protein behaves like a living micro-organism to infect an individual by various routes (even oral administration), survive metabolic clearance, self-replicate in the body, reach the target organ and induce a cascade of neurodegenerative damage. (eyebulb.com)

An emerging wildlife epizootic of chronic wasting disease (CWD) ( 1 ), a contagious prion disease among mule deer, white-tailed deer, and Rocky Mountain elk, has potential public health implications ( 2 - 5 ). (cdc.gov)

Stanley B. Prusiner of the University of California, San Francisco announced in 1982 that his team had purified infectious material and that the infectious agent consisted mainly of a specific protein , although they had not managed to satisfactorily isolate the protein until two years after making his announcement. (wikidoc.org)

Prusiner found that PrpSc molecules can convert Prpc molecules into additional PrpSc molecules, the mechanism that accounts for the infectious nature of these diseases. (wolffund.org.il)
These diseases involve conversion of normal cellular prion protein (PrPc) into a form that is insoluble and resistant to proteases (PrPSc). (ecmbio.com)
Phosphorylation of PrPC at Ser-43 by Cdk5 promotes proteinase K resistance, prion aggregation, and fibril formation in vitro. (ecmbio.com)

Common to all forms of prions is the ability to form highly ordered protein aggregates, so-called amyloid ﬁbrils. (pdf-archive.com)

3 A prion-like propagation process implies that the abnormal protein spreads to involve other nearby cells, whether neuronal or glial. (bmj.com)
On the basis of previous studies on yeast prions that correlated high intrinsic fragmentation rates of ﬁbrils with prion propagation eﬃciency, it has been hypothesized that the nanomechanical properties of prion amyloid such as strength and elastic modulus may be the distinguishing feature. (pdf-archive.com)
However, more recent studies with MSA-derived α-synuclein aggregates have shown that they have a similar ability to undergo template-directed propagation, like PrP prions. (brainsupportnetwork.org)

The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. (cdc.gov)
We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). (cdc.gov)
In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. (cdc.gov)
Outbreaks of prion disease include an epidemic of kuru among the cannibalistic Fore tribe of the New Guinea highlands ( 9 ) and an epizootic of BSE in the United Kingdom, caused by feeding to cattle protein supplements derived from prion-infected cattle offal ( 10 ). (cdc.gov)
As yet, no cases of human prion disease have been linked with CWD ( 5 , 13 - 15 ), and natural transmission of CWD to humans or traditional domestic livestock seems unlikely ( 2 , 3 , 5 , 12 , 14 , 16 , 17 ). (cdc.gov)
The infectious agent in the prion disease is composed mainly or entirely of an abnormal conformation of a host-encoded glycoprotein called the prion protein. (medscape.com)
Spongiform change in prion disease. (medscape.com)
[3] The proteinopathies include such diseases as Creutzfeldt-Jakob disease and other prion diseases , Alzheimer's disease , Parkinson's disease , amyloidosis , multiple system atrophy , and a wide range of other disorders. (wikipedia.org)
[20] They have been most thoroughly studied with regard to prion disease , and are referred to as protein strains . (wikipedia.org)
Other prions cause human illnesses such as Creutzfeld-Jakob disease , a malady that progresses similarly to accelerated Alzheimer's. (theconversation.com)
Bovine spongiform encephalopathy , popularly known as "mad cow disease," is a prion disease that infects cattle. (theconversation.com)
Therefore, the demand on genotyping capacities of the polymorphisms of the prion protein gene ( prnp ) relevant to presently known disease associations and EU regulations is steadily increasing. (biomedcentral.com)
In humans, prions can cause Creutzfeldt-Jakob disease (CJD), a human spongiform encephalopathy whose clinical picture can involve weekly deterioration into blindness and epilepsy as one's brain becomes riddled with tiny holes. (curezone.com)
Adam Renslo and colleagues, who include Nobel Laureate Stanley B. Prusiner, explain that prion diseases include conditions like mad cow disease in animals and Creutzfeldt-Jakob Disease in humans, result from deposits of abnormal prion protein in brain tissue. (scienceblog.com)
But prions can fold in an aberrant shape to become a rogue agent, forcing normal prions to change into the disease-causing shape. (eyebulb.com)
Who discovered prion disease? (eyebulb.com)
When was the first prion disease discovered? (eyebulb.com)
Prusiner received the Albert Lasker Basic Medical Research Award (1994) and the Louisa Gross Horowitz Prize (1997) for his discoveries pertaining to neurodegenerative disease. (eyebulb.com)
Cali I , Puoti G , Smucny J, Curtiss PM, Cracco L, Kitamoto T, Occhipinti R, Cohen ML , Appleby BS, Gambetti P . Co-existence of PrP types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics. (academictree.org)
Cracco L, Xiao X, Nemani SK, Lavrich J, Cali I , Ghetti B, Notari S , Surewicz WK , Gambetti P . Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments. (academictree.org)
This fatal neurodegenerative disease results from misfolding of the normal cellular prion protein (PrP C ) to a pathogenic prion protein form (PrP Sc ). (biomedcentral.com)
CWD(Chronic Wasting Disease)는 매우 전염성이 높은 프리온 질환(prion disease)으로 엘크(elk)와 무스(moose)와 같은 사슴과의 동물들에게서 신경 퇴행성을 유발하는 질환이다. (natureasia.com)
Some UCSF folks continue to say that multiple system atrophy is a prion disease, which means that it's infectious if brain tissue is touched. (brainsupportnetwork.org)
In this review, we discuss the in vitro and in vivo data supporting the recent classification of MSA as a prion disease. (brainsupportnetwork.org)
Neurodegeneration and Prion Disease ed. (aigss-it.org)

Humans and animals can acquire TSEs by consuming prion-contaminated food. (cdc.gov)
The prion diseases are a large group of related neurodegenerative conditions, which affect both animals and humans. (medscape.com)
Amyloid plaques in the brains of animals and humans dying of prion diseases are composed of this abnormal protein. (wolffund.org.il)

α-Synuclein: Multiple System Atrophy Prions. (brainsupportnetwork.org)

Your Majesties, Your Royal Highnesses, Ladies and Gentlemen, This year's Nobel Prize in Physiology or Medicine has been awarded to Stanley B. Prusiner for his discovery of prions - a new biological principle of infection. (eyebulb.com)
Who won the Nobel Prize for the discovery of prions? (eyebulb.com)

In prion diseases, a normally produced cellular protein accumulates in an abnormal, misfolded, and aggregated form ( 6 ), which results in neuron destruction and a universally fatal outcome after a prolonged incubation period. (cdc.gov)
The human cellular prion protein (PrP C ) is a glycosylphosphatidylinositol (GPI) anchored membrane glycoprotein with two N-glycosylation sites at residues 181 and 197. (mdpi.com)
The processing of APP has been extensively studied, while the identity of the cellular proteases involved in the proteolysis of PrP C and their possible role in prion biology has remained limited and controversial. (mdpi.com)
PrP C , the cellular isoform of the prion protein, serves to transduce the neurotoxic effects of PrP Sc , the infectious isoform, but how this occurs is mysterious. (elifesciences.org)
These diseases are caused by refolding of the cellular prion protein (PrP C ) into an infectious isoform (PrP Sc ) that catalytically templates its abnormal conformation onto additional molecules of PrP C ( Prusiner, 1998 ). (elifesciences.org)

B r a prion betegs gek (spongiform encephalopathi k) m r r g ta ismert k rk pek, a betegs gcsoport csak az ut bbi vekben az angliai bovin spongiform encephalopathia (BSE) j rv ny s az els bizony tottan llati eredet hum n prion fert z sek felt n se ut n ker lt a figyelem el ter be. (oszk.hu)

This sequence has high homology to the conserved site in rat, mouse, and bovine prion protein. (ecmbio.com)

Rodent bioassays can be considered a definitive technique for specific detection of prion infectivity. (biomedcentral.com)

ABSTRACT Self-templated protein aggregation and intracerebral deposi- tion of aggregates, sometimes in the form of amyloid ﬁbrils, is a hallmark of mammalian prion diseases. (pdf-archive.com)
Here, we reveal that ﬁbrils formed by mammalian prions are relatively soft and clearly in a diﬀerent class of rigidities when compared to nanoﬁbrils formed by nonprions. (pdf-archive.com)

According to Prusiner's theory, the prion differs from other well-known infections agents including bacteria and viruses. (jrank.org)
You may find answers to your questions if you read up on Stanley Prusiner's (UC-Berkeley) work over the past 30 years or so on prions. (bio.net)

Western blot of GST recombinant human full-length prion protein that was untreated (lanes 1 and 3) or phosphorylated with Cdk5/p25 (lanes 2 & 4). (ecmbio.com)
The antibody detects a human recombinant Prion protein after phosphorylation by Cdk5/p25 complex. (ecmbio.com)

[6] Prusiner coined the word "prion" as a name for the infectious agent, by combining the first two syllables of the words proteinaceous and infectious . (wikidoc.org)

Both the existence of the prion and the underlying mode of infection are unprecedented in medical sciences. (jrank.org)
[2] In general usage, prion can refer to both the theoretical unit of infection or the specific protein (e.g. (wikidoc.org)

All known prions induce the formation of an amyloid fold, in which the protein polymerizes into a fiber with a core consisting of tightly packed beta sheets . (wikidoc.org)

When did Robert Prusiner win the Horowitz Prize? (eyebulb.com)

In Prion Biology and Diseases. (ac.be)

He answered many of these question through work on the sheep variety of prion that has been around for a long time. (bio.net)

The reference to Prusiner brought back memory of his article which appeared in Science in 1982 where a lot is said about the resistance of prion to a number of things. (bio.net)
Prusiner, S.B. (1982) Science. (ecmbio.com)

Wickner (1994) discovered that a yeast nonchromosomal genetic element, [URE3], was an altered form of the yeast protein, Ure2p and proposed it to be a yeast prion. (eyebulb.com)

Prions are believed to infect and propagate by refolding abnormally into a structure which is able to convert normal molecules of the protein into the abnormally structured form. (wikidoc.org)

Prion diseases can result from modification of a host-encoded glycoprotein (i.e. (wikidoc.org)

Prions are associated with a unique protein found in brain tissue called prion protein (PrP). (wustl.edu)

A better understanding of the role of PrP in prion diseases may help to reveal new treatments for these and other degenerative brain disorders. (elifesciences.org)
Prion diseases are invariably fatal and no treatments are yet available. (scienceblog.com)
The compounds appear to be among the most promising potential treatments for prion diseases yet discovered, the report suggests. (scienceblog.com)

Major prion protein (PrP) is encoded in the human body by the PRNP gene also known as CD230 (cluster of differentiation 230). (wikipedia.org)
In vitro studies ( 11 , 12 ) indicate that this natural barrier reduces human susceptibility to animal prion diseases, including CWD. (cdc.gov)
PRNP ( PR io N P rotein) is the human gene encoding for the major prion protein PrP (for pr ion p rotein), also known as CD230 ( cluster of differentiation 230). (wikidoc.org)
Endogenous prion phosphorylation was examined in human PC3 cells untreated (lanes 5 & 7) or treated with Calyculin A (100 nM) for 30 min (lanes 6 & 8). (ecmbio.com)
Prion Protein (Ser-43) antibody was generated from a phospho-peptide that included amino acids surrounding Serine 43 in human prion protein. (ecmbio.com)

After purifying prions from the brain, he discovered that they are composed of a special type of protein encoded by a chromosomal gene. (wolffund.org.il)
He identified the gene behind the prion protein, but determined that it is also present in healthy people and animals. (eyebulb.com)

Prion diseases are always fatal , but they don't all affect the same species. (theconversation.com)
All known prion diseases affect the structure of the brain or other neural tissue, and all are currently untreatable and fatal. (wikidoc.org)

Prion diseases are a group of degenerative illnesses of the brain caused when a molecule called the prion protein (PrP for short) adopts the wrong shape. (elifesciences.org)
Tests on prion-infected mouse brain cells showed that the compounds reduced the amount of the abnormal prion protein. (scienceblog.com)

Two in vitro detection techniques such as protein misfolding cyclic amplification (PMCA) and real-time quaking-induced conversion (RT-QuIC) have demonstrated improved prion detection in PrP Sc infected tissues. (biomedcentral.com)

These findings and others resulted in the theory that PD may be a prion-related disorder, yet some of the criteria fall short for the full definition [ 16 , 17 ]. (nature.com)

Food-based prion transmission between species also occurs, although a phenomenon known as the "species barrier" decreases transmission efficiency. (cdc.gov)

Morphological characteristics of prion nanoﬁbrils. (pdf-archive.com)

Protocols for prion purification allows us to detect and characterize prion in a complex mixture with inhibitors and/or in a diluted samples to improve PrP Sc detection sensitivity. (biomedcentral.com)

The CWD prion favors cervids, or deerlike animals . (theconversation.com)
The protein that prions are made of is found throughout the body, even in healthy people and animals. (wikidoc.org)

[ 3 ] Accidental transmission of prions is a recurrent event in the history of these agents and is related to their unusual biophysical properties. (medscape.com)

Prion protein contains five octapeptide repeats with sequence PHGGGWGQ (though the first repeat has the slightly-modified, histidine-deficient sequence PQGGGGWGQ). (wikipedia.org)
Prion protein contains 5 amino-terminal octapeptide repeats with sequence PHGGGWGQ. (wikidoc.org)

The early positive signs for so-called prion diseases come from research in laboratory mice and cell cultures, they say in a report in ACS' Journal of Medicinal Chemistry. (scienceblog.com)

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