Exact(1)
Neuronal ceroid lipofuscinoses (NCLs) comprise a heterogeneous group of metabolic storage diseases that present with the accumulation of autofluorescent lipopigment, neurodegeneration and premature death.
Similar(58)
Of note, Vps15-deficient muscles, but not Atg7-deficient muscles, presented with the accumulation of all above-mentioned proteins in the vacuoles within the fibres (Fig. 8D and Supporting Information Fig. 11A).
The effects of depletion of VPS20/CHMP6 seen in the present study, with the accumulation of tethered MVBs associated with swollen LAMP1 positive endocytic compartments, are also consistent with a regulatory role in endosome lysosome fusion, although they could equally imply that all ubiquitinated cargoes must be correctly sorted into MVBs if they are to become competent for fusion.
However, it is not clear from the present data if the accumulation of acetate with the concomitant generation of NAD(P H results in response to an increased NAD(P H demand for HMF and furfural conversion, or if it results from acetaldehyde accumulation due to the competitive inhibition of alcohol dehydrogenases by the inhibitors.
The authors cite the Mexican intellectual Carlos Fuentes, who has said that the present is merely the accumulation of frustrated goals of the past: "There is no single time: all of our times are alive, all of our pasts are present".
In the present study, the expressions of Unigene 0016938 and Unigene 001639 were not paralleled with the accumulation of anthocyanins.
There are opportunities and risks with the accumulation of data, just as there are for the accumulation of capital.
How could virtue be reconciled with the accumulation of unnecessary objects?
As of this evening, with the accumulation of absentee ballots, Mr. Bush's lead in Florida was 930 votes.
"People are no longer obsessed with the accumulation of things," Picard tells him — and us, the audience — sternly.
The ultrastructural examination of the large neuronal inclusions indicates that they are derived from the deposition and build-up of lysosomal components, as is characteristically seen in lysosomal storage disorders which typically present with an accumulation of autofluorescent material [ 31].
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