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In glycogen-storage diseases glycogen cannot be metabolized to yield lactic acid and energy, so that it accumulates within muscle, liver, and other tissues.
To understand the mechanism of the liver disease associated with ATD, our laboratory focused initially on mechanisms by which mutant ATZ is degraded when it accumulates within cells.
The truncated protein lacks the zinc finger domain and should fail to undergo nuclear import (Bedard et al., 2007); however, it accumulates within the nucleus as a result of passive diffusion.
These results suggest that the mutant fibrinogen Aguadilla behaves similarly to mutant ATZ in the fact that it accumulates within the secretory pathway, has a tendency to polymerize/aggregate, and is degraded by proteasomal and autophagic mechanisms.
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In these cells, it accumulated within the lysosomal compartment and enhanced zinc-inducible IFNγ production.
Most significantly, it accumulates specifically within the BS chloroplasts of maize and other C4 plants.
However, production of yolk continues, and consequently it accumulates in large pools within the body cavity.
In the study reported here we show that uptake of rucaparib is carrier mediated, it accumulates and is retained within cancer cells and tumour xenograft but not brain tissues.
Extracellular ATP is generally considered the prototypical danger signal, as it accumulates at inflammatory sites and within the tumor interstitium at concentrations that may reach the hundred micromolar range.
Disruption of 14-3-3 14-3-3 14-3-3y mutatinteractionand/or Ser to an alanine residue does not affect LRRK2 kinase activity byt has an imutation LRRK2 lofalization, cauSerg it to and/orlate within cytoplaSerc pools instoan of being diffusely localaninethresiduet the cytoplasm [ 6].
The product CaS accumulates within the particles.
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