Brain Science Glossary

What are prions? Misfolded proteins? Or abeta, tau and synuclein? No, these aren’t exotic pizza toppings, silly! Learn about the basic terms of brain science here.

Prions (aka Misfolded Proteins)

Most proteins in the body are evolutionary designed to fold into a dominant three-dimensional shape that facilitates its structural and functional role. Occasionally, proteins may misfold into non-functional or even toxic shapes. These toxic misfolded proteins are referred to as Prions. Prions are most stable in the form of aggregates

Prion Aggregates

A very small number of proteins can misfold into stable clusters, known as aggregates. Abeta, Tau, and Synuclein are three proteins that can misfold into self-replicating aggregates known as Prion Aggregates.

Characteristics of Prions

Prions are toxic, misfolded protein aggregates that replicate in the body by causing normal proteins to misfold and form more Prions. Prions are believed to be the active form of many neurodegenerative diseases, including Alzheimer’s and Parkinson’s and are responsible for progression and spread of these diseases within the brain.

Abeta Amyloid or Abeta for short

Abeta is a normal brain protein that may misfold and form toxic self-replicating Prions in Alzheimer’s patients. At late stages of Alzheimer’s, large misfolded aggregates of Abeta Amyloid accumulate in the brain and are referred to as plaques

Tau

Tau is a second normal brain protein that may misfold and form toxic, self-replicating Prions in patients with Alzheimer’s disease and other neurodegenerative diseases. At late stages of Alzheimer’s, large misfolded aggregates of Tau accumulate within nerve cells and are referred to as neurofibrillary tangles, or Tangles for short. 

Alpha Synuclein or Synuclein for short

Synuclein is a third normal brain protein that may misfold and form toxic, self-replicating Prions in patients with Parkinson’s disease and other neurodegenerative diseases affecting movement. At late stages of Parkinson’s, large misfolded aggregates of Synuclein accumulate within nerve cells and are referred to as Lewy Bodies

Plaques

Plaques refer to large misfolded aggregates of Abeta Amyloid that accumulate in the brain. Plaques are detectable only under the microscope.

Tangles

Neurofibrillary tangles, or Tangles for short. They are large misfolded aggregates of Tau that accumulate within nerve cells. Tangles are detectable only under the microscope.

Lewy Bodies

Lewy Bodies are large misfolded aggregates of Synuclein that accumulate within nerve cells at late stages of Parkinson’s. Lewy Bodies are detectable only under the microscope.

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