Huntingtin Protein and Protein Aggregation
Part 6

What Causes the Onset of HD?

Altered Huntingtin and the Ubiquitin-Proteasome System

Normal cells have a mechanism for quality control called the ubiquitin-proteasome system (UPS). The UPS is a protein-chaperone system which tags misfolded or damaged proteins for re-folding, or more commonly, for degradation. In contrast, protein aggregates resulting from the altered HD gene have a high molecular mass and are sequestered to the aggresome region of a cell. (See Figure P-3.)

Last Modified: 04/12/2007

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