Aggresomes, inclusion bodies and protein aggregation - PubMed (original) (raw)
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Aggresomes, inclusion bodies and protein aggregation
R R Kopito. Trends Cell Biol. 2000 Dec.
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Abstract
Intracellular and extracellular accumulation of aggregated protein are linked to many diseases, including ageing-related neurodegeneration and systemic amyloidosis. Cells avoid accumulating potentially toxic aggregates by mechanisms including the suppression of aggregate formation by molecular chaperones and the degradation of misfolded proteins by proteasomes. Once formed, aggregates tend to be refractory to proteolysis and to accumulate in inclusion bodies. This accumulation has been assumed to be a diffusion-limited process, but recent studies suggest that, in animal cells, aggregated proteins are specifically delivered to inclusion bodies by dynein-dependent retrograde transport on microtubules. This microtubule-dependent inclusion body is called an aggresome.
Comment in
- Ron R. Kopito: Unfolding the Secrets of Protein Aggregation.
Kopito RR. Kopito RR. Trends Cell Biol. 2016 Aug;26(8):559-560. doi: 10.1016/j.tcb.2016.05.001. Epub 2016 May 27. Trends Cell Biol. 2016. PMID: 27238420 No abstract available.
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