creutzfeldt-jakob disease (cjd)
... Family of progressive neurodegenerative disorders. Affect both humans and animals. Long incubation periods. A unique feature of these diseases is that they can be inherited, arise spontaneously or may be acquired through infection. Characteristic neuropathologic feature of multifocal spongiform chan ...
... Family of progressive neurodegenerative disorders. Affect both humans and animals. Long incubation periods. A unique feature of these diseases is that they can be inherited, arise spontaneously or may be acquired through infection. Characteristic neuropathologic feature of multifocal spongiform chan ...
Creutzfelt-Jacob Disease
... • Some cases of CJD are transmitted by exposure to prioncontaminated instruments, tissues, serum, hormones, etc, from individuals with CJD - ‘known or suspected source’ ...
... • Some cases of CJD are transmitted by exposure to prioncontaminated instruments, tissues, serum, hormones, etc, from individuals with CJD - ‘known or suspected source’ ...
Essential Forces in Protein Folding
... recognition by interacting with molecules such as substrates, receptors, signaling proteins and cell surface adhesion molecules. • When proteins do not fold correctly (misfolding) there can be serious health consequences, including many well known diseases, such as Alzheimer's, Mad Cow (Bovine spong ...
... recognition by interacting with molecules such as substrates, receptors, signaling proteins and cell surface adhesion molecules. • When proteins do not fold correctly (misfolding) there can be serious health consequences, including many well known diseases, such as Alzheimer's, Mad Cow (Bovine spong ...
Transmission of Prions
... The conformational hypothesis proposes that each strain is associated with a different conformation of PrPSc and that each can convert the PrPC of its host into a likeness of itself. Indeed, PrPSc species associated with 2 hamster-adapted scrapie strains, HY and DY, are cleaved to products of differ ...
... The conformational hypothesis proposes that each strain is associated with a different conformation of PrPSc and that each can convert the PrPC of its host into a likeness of itself. Indeed, PrPSc species associated with 2 hamster-adapted scrapie strains, HY and DY, are cleaved to products of differ ...
Lh6Ch04bProt
... 1. Know collagen structure and the role of vitamin C. 2. Structure of globular proteins, circular dichroism, and the main idea of protein families (there are over 800). 3. Denaturation and Renaturation (or not) of proteins 4. One of the largest unsolved puzzles in modern biochemistry: the details of ...
... 1. Know collagen structure and the role of vitamin C. 2. Structure of globular proteins, circular dichroism, and the main idea of protein families (there are over 800). 3. Denaturation and Renaturation (or not) of proteins 4. One of the largest unsolved puzzles in modern biochemistry: the details of ...
Fundamentals of prions and their inactivation (Review)
... in host cells, especially neurons, and has possible functions including the regulation of anti-oxidative stress, calcium and copper homeostasis, circadian rhythms, spatial learning, memory, signalling, and synapse formation (16,17), changes into PrPSc (Fig. 2). PrPSc accumulates in the brain and for ...
... in host cells, especially neurons, and has possible functions including the regulation of anti-oxidative stress, calcium and copper homeostasis, circadian rhythms, spatial learning, memory, signalling, and synapse formation (16,17), changes into PrPSc (Fig. 2). PrPSc accumulates in the brain and for ...
Studying Viruses and Prokaryotes
... You are probably familiar with the terms virus and bacteria, but you may not know exactly what they are. A virus is an infectious particle made only of a strand of DNA or RNA surrounded by a protein coat. Bacteria, on the other hand, are one-celled microorganisms that can also cause infection. Any l ...
... You are probably familiar with the terms virus and bacteria, but you may not know exactly what they are. A virus is an infectious particle made only of a strand of DNA or RNA surrounded by a protein coat. Bacteria, on the other hand, are one-celled microorganisms that can also cause infection. Any l ...
Unusual infectious agents
... • Familial (genetic) spongiform encephalopathy • Sporadic spongiform encephalopathy • All three diseases can be transmitted ...
... • Familial (genetic) spongiform encephalopathy • Sporadic spongiform encephalopathy • All three diseases can be transmitted ...
ppt - IPAW
... certain degenerative neurological diseases, such as sheep scrapie and bovine spongiform encephalopathy (BSE), and the human dementias Creutzfeldt-Jacob disease (CJD) and Gerstmann-Straussler syndrome (GSS). PrP is encoded in the host genome and is expressed both in normal and infected cells. During ...
... certain degenerative neurological diseases, such as sheep scrapie and bovine spongiform encephalopathy (BSE), and the human dementias Creutzfeldt-Jacob disease (CJD) and Gerstmann-Straussler syndrome (GSS). PrP is encoded in the host genome and is expressed both in normal and infected cells. During ...
Viruses - mbhsbiology
... What is a Prion? A prion is a ________________ that can cause infection or disease Prions are different from viruses in their composition: - prions consist of protein _____________ while viruses contain nucleic acids and a protein coat And how they replicate: – When mutated prions comes in contact w ...
... What is a Prion? A prion is a ________________ that can cause infection or disease Prions are different from viruses in their composition: - prions consist of protein _____________ while viruses contain nucleic acids and a protein coat And how they replicate: – When mutated prions comes in contact w ...
Prions, viral pathogens
... What was the controversy about publishing mutations that made avian influenza able to better infect human cells? What are some reasons influenza could be used as a bioweapon? ...
... What was the controversy about publishing mutations that made avian influenza able to better infect human cells? What are some reasons influenza could be used as a bioweapon? ...
Viruses - NIU Department of Biological Sciences
... have no DNA or RNA. The main protein involved in human and mammalian prion diseases is called “PrP”. Similar proteins have been found in other organisms, including some with useful, positive effects. (For the literary types: prions work approximately like “ice-9” did in the novel Cat’s Cradle, by Ku ...
... have no DNA or RNA. The main protein involved in human and mammalian prion diseases is called “PrP”. Similar proteins have been found in other organisms, including some with useful, positive effects. (For the literary types: prions work approximately like “ice-9” did in the novel Cat’s Cradle, by Ku ...
Viruses
... have no DNA or RNA. The main protein involved in human and mammalian prion diseases is called “PrP”. Similar proteins have been found in other organisms, including some with useful, positive effects. (For the literary types: prions work approximately like “ice-9” did in the novel Cat’s Cradle, by Ku ...
... have no DNA or RNA. The main protein involved in human and mammalian prion diseases is called “PrP”. Similar proteins have been found in other organisms, including some with useful, positive effects. (For the literary types: prions work approximately like “ice-9” did in the novel Cat’s Cradle, by Ku ...
viewpoint - Lindquist Lab
... hereditary information, termed ‘genes’ by the Danish botanist Wilhelm Johannsen in 1909, revealed that they comprise nucleic acids, and was first published by Oswald Avery and colleagues in 1944 (Avery et al, 1944). The seminal paper on the doublehelical structure of DNA by James Watson and Francis ...
... hereditary information, termed ‘genes’ by the Danish botanist Wilhelm Johannsen in 1909, revealed that they comprise nucleic acids, and was first published by Oswald Avery and colleagues in 1944 (Avery et al, 1944). The seminal paper on the doublehelical structure of DNA by James Watson and Francis ...
Chronic Wasting Disease (CWD)
... Prions are proteinacious infectious agents causing transmissible spongiform encephalopathies (TSEs): a group of neurodegenerative diseases that includes in humans kuru, Creutzfeldt–Jakob Disease (CJD) and variant CJD (vCJD) and in animals scrapie of sheep and goats, bovine spongiform encephalopathy ...
... Prions are proteinacious infectious agents causing transmissible spongiform encephalopathies (TSEs): a group of neurodegenerative diseases that includes in humans kuru, Creutzfeldt–Jakob Disease (CJD) and variant CJD (vCJD) and in animals scrapie of sheep and goats, bovine spongiform encephalopathy ...
Amyloidosis - chem.uwec.edu
... hosts, e.g. CWD, BSE, kuru, etc… Amyloid diseases not thought to be infectious agents but…. ...
... hosts, e.g. CWD, BSE, kuru, etc… Amyloid diseases not thought to be infectious agents but…. ...
Protein Misfolding Can Have Deadly Consequences 992328張謹淳
... • For whatever reason, the brain of aging mice show no evidence of the amyloid deposits found in humans, and there was no animal model for AD. • Until 1995, the researchers create a strain of mice by genetically engineering the mice to carry a mutant human APP gene. ...
... • For whatever reason, the brain of aging mice show no evidence of the amyloid deposits found in humans, and there was no animal model for AD. • Until 1995, the researchers create a strain of mice by genetically engineering the mice to carry a mutant human APP gene. ...
Tinkering with the Biochemistry of Life: Viruses, Prions, and Peptide
... 2. Determine which targets can be disabled 3. Design chemical that inhibits target Similarly, body recognizes target protein antigens, mounts defense based on antigen recognition. ...
... 2. Determine which targets can be disabled 3. Design chemical that inhibits target Similarly, body recognizes target protein antigens, mounts defense based on antigen recognition. ...
ADH | CWD Fact Sheet - Arkansas Game and Fish Commission
... American cervids (mule deer, white-tailed deer, elk, and moose). Prion proteins are found in the brains of normal healthy animals. However, the functions of these normal prion proteins are still not completely understood. Prion disease occurs when the normal prion proteins fold abnormally which lead ...
... American cervids (mule deer, white-tailed deer, elk, and moose). Prion proteins are found in the brains of normal healthy animals. However, the functions of these normal prion proteins are still not completely understood. Prion disease occurs when the normal prion proteins fold abnormally which lead ...
Prions and the like
... Charles Weissmann and colleagues showed that mice with inactivated PRNP remained well when infected with scrapie, whereas scrapie-infected mice with PRNP died. It appears that any organism lacking PrPC will be resistant to prion infection. Sebastian Brandner and Adriano Aguzzi helped to establish th ...
... Charles Weissmann and colleagues showed that mice with inactivated PRNP remained well when infected with scrapie, whereas scrapie-infected mice with PRNP died. It appears that any organism lacking PrPC will be resistant to prion infection. Sebastian Brandner and Adriano Aguzzi helped to establish th ...
PROTEIN MISFOLDING
... proteins to join the aggregation process and adopt the abnormal conformation • In a sort of cascading "domino effect", a high concentration of abnormally folded protein (PrPSC) forms amyloid plaques (large insoluble fibrous aggregates with β conformation prevalent) in brain. ...
... proteins to join the aggregation process and adopt the abnormal conformation • In a sort of cascading "domino effect", a high concentration of abnormally folded protein (PrPSC) forms amyloid plaques (large insoluble fibrous aggregates with β conformation prevalent) in brain. ...
Mad Cow Disease
... The causative agent for Mad Cow disease are prions. A prion is a nonliving, self-replicating infectious agent made of protein. It can replicate with the aid of its host, similarly to what a virus does. Prion is short for “proteinaceous infectious particle.” Prions carry the disease between individua ...
... The causative agent for Mad Cow disease are prions. A prion is a nonliving, self-replicating infectious agent made of protein. It can replicate with the aid of its host, similarly to what a virus does. Prion is short for “proteinaceous infectious particle.” Prions carry the disease between individua ...
Prion
A prion (/ˈpriːɒn/) is a protein that can fold in multiple, structurally distinct ways, at least one of which is transmissible to other prion proteins. It is this form of replication that leads to disease that is similar to viral infection. The word prion, coined in 1982 by Stanley B. Prusiner, is short for “proteinaceous infectious particle” derived from the words protein and infection, in reference to a prion's ability to self-propagate and transmit its conformation to other prions. While several yeast proteins have been identified as having prionogenic properties, the first prion protein was discovered in mammals and is referred to as the major prion protein (PrP). This infectious agent causes mammalian transmissible spongiform encephalopathies, including bovine spongiform encephalopathy (BSE, also known as ""mad cow disease"") and scrapie in sheep. In humans, PrP causes Creutzfeldt-Jakob Disease (CJD), variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann–Sträussler–Scheinker syndrome, Fatal Familial Insomnia and kuru.A protein as an infectious agent stands in contrast to all other known infectious agents, like viruses, bacteria, fungi, or parasites—all of which must contain nucleic acids (either DNA, RNA, or both). All known prion diseases in mammals affect the structure of the brain or other neural tissue and all are currently untreatable and universally fatal.Prions are not considered living organisms because they are misfolded protein molecules which may propagate by transmitting a misfolded protein state. If a prion enters a healthy organism, it induces existing, properly folded proteins to convert into the misfolded prion form. In this way, the prion acts as a template to guide the misfolding of more proteins into prion form. In yeast, this refolding is assisted by chaperone proteins such as Hsp104p. These refolded prions can then go on to convert more proteins themselves, leading to a chain reaction resulting in large amounts of the prion form. All known prions induce the formation of an amyloid fold, in which the protein polymerises into an aggregate consisting of tightly packed beta sheets. Amyloid aggregates are fibrils, growing at their ends, and replicate when breakage causes two growing ends to become four growing ends. The incubation period of prion diseases is determined by the exponential growth rate associated with prion replication, which is a balance between the linear growth and the breakage of aggregates. (Note that the propagation of the prion depends on the presence of normally folded protein in which the prion can induce misfolding; animals that do not express the normal form of the prion protein can neither develop nor transmit the disease.)Prion aggregates are extremely stable and accumulate in infected tissue, causing tissue damage and cell death. This structural stability means that prions are resistant to denaturation by chemical and physical agents, making disposal and containment of these particles difficult. Prion structure varies slightly between species, but nonetheless prion replication is subject to occasional epimutation and natural selection just like other forms of replication.