The phrase
"aggregated proteins" refers to proteins that have clumped together or formed clusters.
Full definition
A common feature of neurodegenerative diseases such as Alzheimer's, Parkinson's or Huntington's disease are deposits
of aggregated proteins in the patient's cells that cause damage to cellular functions.
Several human neurodegenerative diseases, including Alzheimer's, Parkinson's and Huntington's disease but also aging, are linked to an accumulation of abnormal and
aggregated proteins in cells.
Huntington's disease, for example, is caused
by aggregating proteins inside brain neurons that ultimately lead to motor dysfunction, personality changes, depression and dementia, usually progressing rapidly after onset in people's 40s.
«Much to our surprise, we found that even the most
severely aggregated proteins disassembled and went back into circulation during recovery.
Remarkably, this pathway seems specific
for aggregated proteins like huntingtin and appears to be more potent than previously discovered cellular garbage disposal mechanisms.
Optineurin is involved, for example, in the elimination
of aggregated proteins and bacterial infection defense.
Proteasomes are cytosolic molecular machines involved in the degradation of misfolded and
aggregated proteins.
Researchers assumed that the drug prevented the neurodegeneration seen in Alzheimer's by encouraging cells to degrade damaged components and
aggregated proteins.
Aggregated proteins are potential candidates for causing age - related dementia.
They traced the effect to increased production of a specific type of lipid that, surprisingly, prevented the formation of
aggregate proteins.
In their work, the scientists sought to create, and then manipulate the shape of, amyloid fibrils — rods of
aggregated proteins, or peptides, that match the strength of spider's silk.
«The assumption has been that the disease is caused by
the aggregated proteins,» Workman says.
«But in the mutated fly, there's
no aggregated protein.
«Hopefully with a combination of biochemistry and genetics we can sort out how much of the neurodegeneration comes from the fact that you don't have the normal Ataxin - 7 protein, and how much is because of
the aggregated protein,» Abmayr says.
Patrik Verstreken (VIB / KU Leuven): «We have set up a new in vitro system specifically for this research, to enable us to study the distribution throughout the brain of
aggregate proteins that are typical of neurodegenerative diseases.
Reporting in Cell on Sept. 10, 2015, scientists from the University of Chicago and Harvard University discovered that aggregates are fully reversible — after the cell returns to normal temperatures,
aggregated proteins are disentangled and resume their normal cellular functions.
The biggest surprise came when the team looked at the fates of
aggregated proteins.
They found that
aggregated proteins were disentangled and resumed their original functions without exception after cells returned to normal temperatures.
Isotope labeling ruled out the possibility that
aggregated proteins were being degraded and replaced by new proteins, which would be unlabeled.
That finding reversed a common dogma that misfolded and
aggregated proteins are irredeemable, Lindquist said.
Weeks later, where the vector was applied,
aggregated proteins had almost disappeared.
We hypothesize that
these aggregating proteins also block the transport of normally transported axonal proteins, and thus cause neurodegeneration and neuronal death by impairing axonal transport.
On the one hand, autophagy functions as a tumor suppressor mechanism by preventing the accumulation of damaged organelles and
aggregated proteins.
Autophagy also plays a housekeeping role in removing misfolded or
aggregated proteins, clearing damaged organelles, such as mitochondria, endoplasmic reticulum and peroxisomes, as well as eliminating intracellular pathogens.
It stimulates autophagy, a cellular mechanism that recycles unneeded, misfolded, and
aggregated proteins, reducing their accumulation associated with aging.
The enzyme might attach to
aggregated protein and by straightening the proline bend, it helps unstack the amino acid chain.