Lecture on Prions and Transmissible Spongiform Encephalopathies

Flashcards covering key vocabulary and concepts related to prions and transmissible spongiform encephalopathies.

Prions

Infectious misfolded proteins that cause neurodegenerative diseases.

Transmissible spongiform encephalopathy (TSE)

A class of neurodegenerative diseases characterized by sponge-like degeneration of the brain.

Kuru

A human TSE first identified in Papua New Guinea, transmitted through ritual cannibalism.

Creutzfeldt-Jakob disease (CJD)

A degenerative brain disorder, often sporadic, familial, or acquired.

Bovine spongiform encephalopathy (BSE)

Also known as 'mad cow disease', a TSE that affects cattle and can infect humans.

Familial TSE

A form of TSE that occurs due to mutations in the prion protein gene.

Acquired TSE

A form of TSE caused by consumption of infected prion proteins.

PrP

A prion protein that exists in a normal (PrPc) and a misfolded (PrPsc) form.

Prion fibrils

Long aggregates of misfolded prion proteins that are resistant to proteolysis.

Alpha helix and beta sheet

Secondary structures that play roles in protein folding, with prions exhibiting a shift from alpha helices to beta sheets.

Heat shock proteins (HSPs)

Chaperone proteins that assist in protein folding and prevent misfolding.

Endocytosis

The process by which cells internalize substances, including prions.

Trans-synaptic transmission

The process through which prions can spread from cell to cell.

Neurodegeneration

The progressive loss of structure or function of neurons.

Incubation period

The time between exposure to the prion and the onset of symptoms.

Toxicity of prions

The harmful effects of prions leading to cellular dysfunction and neuronal death.

Ritual mortuary cannibalism

The practice leading to transmission of kuru, by consuming deceased relatives.

Sporadic TSE

A TSE that occurs without a known genetic cause or acquired infection.

Neuronal cell death

The loss of neurons which is a consequence of prion accumulation.

Genetic criteria for prions

Characteristics that define a true prion, including heritable properties.

Phenotypic diversity

Variation in phenotype arising without changes in genotype, often seen in prion strains.