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Inefficient degradation can lead to accumulation of protein fragments and subsequent aggregation.
Fenton oxidation sometimes gives inefficient degradation because of iron-organic complexes [11, 12].
Previous studies have shown that deficient autophagy results in a marked increase in HTT protein expression, due to inefficient degradation [24], and blocking autophagy in models of Huntingdon's disease in vitro (in adrenal medulla cells or striatal cells) raised levels of exogenously expressed HTT, reduced cell viability, and increased the number of cells bearing mutant HTT aggregates [14].
The accumulation was most likely due to inefficient degradation of LYL1 by the proteasome.
However, microbes which produce the enzyme in low quantities lead to inefficient degradation of cellulose.
More recently, autophagosome transport was also found defective in primary neurons expressing mHtt, leading to inefficient degradation of engulfed mitochondria.
Similar(43)
Free-hydroxyl phenolic units can decrease or even abort the catalytic activity of lignin peroxidase H8 during oxidation of veratryl alcohol and model lignin dimers, resulting in slow and inefficient lignin degradation.
Renal DNaseI gene shutdown may therefore impose chromatin exposure in situ because of inefficient enzymatic degradation.
Lipofuscin accumulation - a result of inefficient lysosomal degradation - may in turn exacerbate both lysosomal and autophagic abnormalities.
Although T. reesei have been explored extensively for cellulase production, the major limitations are the low β-glucosidase activity and inefficient biomass degradation, respectively.
In addition, results from Gukovskaya's group suggest that inefficient autophagic degradation of zymogens due to defective lysosomal proteolytic activity may promote pancreatitis.
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