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sequestosome


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A perinuclear inclusion body (aggresome) containing aggregated, misfolded, ubiquitinated protein, destined for degradation. May arise if the capacity of the proteosome is exceeded, which may also be the case for Lewy bodies. Cells that over-express wild-type parkin form fewer aggresomes and disruption of microtubules blocks the formation of aggresomes. The sequestosome-1 gene encodes ubiquitin-binding protein p62 and Paget's disease of bone type 3 is due to mutation in this gene.

Subjects: Medicine and Health.


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