Not exact matches
A genetic cure
for the disease would require completely replacing the mutated gene because any remaining dysfunctional
protein would continue to form damaging
aggregates.
Very large
aggregates can be formed from
protein subunits,
for example many thousand actin molecules assemble into an actin filament.
Optineurin is involved,
for example, in the elimination of
aggregated proteins and bacterial infection defense.
Once CAT - tailed
proteins are imported into the mitochondria, they form
aggregates that may act as a seed, and ultimately bind
proteins free of defects that have vital roles
for the cell» explains Toshiaki Izawa, first author of the study, together with Sae - Hun Park.
Hartl has been investigating
protein aggregates, a cellular cause of neurodegenerative diseases,
for many years.
While these compounds did not reduce the amount of
protein aggregates, they were found to reduce the
protein's toxicity and to increase the stability of amyloid fibrils — a finding that lends further evidence to the theory that smaller assemblies of amyloid - beta known as oligomers, and not the fibrils themselves, are the toxic agents responsible
for Alzheimer's symptoms.
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.
Key to this process is a repetitive stretch of a
protein building block called glutamine, which is known to serve as a glue
for protein aggregates in disease.
«We already know from previous studies on the
protein huntingtin, which spontaneously forms
aggregates and is responsible
for the development of the neurodegenerative disorder Huntington's disease, that
protein aggregates also bind to essential
proteins that have no defects.»
The report stated that «there is a reasonable certainty that no harm will result from
aggregate exposure to the United States population, including infants and children, to the CryIA (b)
protein and the genetic material necessary
for its production.
«Our results not only demonstrate a potential mechanism
for the development of neurodegenera - tive disorders but we have also found another example of the way in which
proteins can form
aggregates and damage the cell.
Previously, researchers have shown that treating cells with neuregulin - 1,
for example, dampens levels of amyloid precursor
protein, a molecule that generates amyloid beta, which
aggregate and form plaques in the brains of Alzheimer's patients.
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.
«The TTR
protein has a tendency to misfold and then
aggregate, which
for some reason seems to occur in children with JIA,» said Dr. Santambrogio.
Scientists at the Stowers Institute
for Medical Research have made a surprising finding about the
aggregates of misfolded cellular
proteins that have been linked to aging - related disorders such as Parkinson's disease.
When isolated and tested
for their response to heat, these
proteins readily
aggregated.
For example, it helps to remove
proteins that fail to fold properly, which would otherwise form harmful
aggregates.
A detailed analysis of this mechanism will be crucial to understand how
aggregate - forming
proteins lead to human diseases and may help to develop concepts
for possible disease preventions.
We used this paradigm following the field of neurodegenerative disorders associated to
protein misfolding, in which a widely established method
for studying propagation of
protein aggregates in vitro consists of exposing cell lines to specific
protein aggregates.
The newly identified
proteins, termed CUET
proteins (
for Cue5 / Tollip adaptors; shown in red), recognize pathological
protein aggregates (cellular waste), including those of human neurodegenerative diseases.
For that purpose, we used
aggregates of the Tau
protein, implicated in Alzheimer's disease, as well as the bacterial amyloid
protein microcin (Mcc).
Isolated islets from 3 - wk - old Tg - hIAPP, which do not exhibit IAPP
aggregates at that age (Janson et al., 1996), were cultured in the presence of 1 % or 0.1 % islets extracts from old Tg or WT mice
for 7 d under standard conditions, including a glucose concentration of 11 mM in the medium to reduce glucotoxicity and spontaneous aggregation of the
protein (Zraika et al., 2007).
AEC syndrome caused by pathological
protein aggregates / Research lays groundwork
for causal therapies.
AFM (2015 - 2016): RĂ©gulation of
protein aggregation trough NBR1 and P62 phosphorygation: implication
for protein aggregate myopathies
They also work as garbage collectors, chewing up dead cells and molecular debris strewn among living cells — including clusters of a
protein called A-beta, notorious
for aggregating into gummy deposits called Alzheimer's plaques, the disease's hallmark anatomical feature.
Lysosomes are responsible
for the turnover and clearance from cells of damaged organelles and
protein aggregates.
For studies at the molecular level, Ismagilov will develop microfluidic systems capable of rapidly generating, manipulating and assaying
protein aggregates of various sizes, shapes and compositions.
A comparison of the absorption and corrected excitation spectra of the Amrose
proteins suggests the presence of a certain amount of non-emissive dimers or
aggregates as shown in Figure 3 exemplarily
for Amrose v1, indicated by the mismatch of the signals on their short wavelength side.
The crystallins, which make up over 90 % of the
proteins in the lens, are specially adapted to contribute to the maintenance of transparency by forming soluble, high - molecular weight
aggregates that need to stay in solution
for the duration of an individual's life.