We review the mechanisms responsible for amino acid homeostasis in Saccharomyces
cerevisiae and other fungi. Amino acid homeostasis is essential for cell growth and survival …

Mutations in the CTNS gene encoding the lysosomal membrane cystine transporter
cystinosin are the cause of cystinosis, an autosomal recessive lysosomal storage disease …

Hydrolysis within the vacuole in yeast and the lysosome in mammals is required for the
degradation and recycling of a multitude of substrates, many of which are delivered to the …

The development over the past 50 years of a variety of cell lines and animal models has
provided valuable tools to understand the pathophysiology of nephropathic cystinosis …

Cystinosis is an autosomally inherited rare genetic disorder in which cystine accumulates in
the lysosome. The defect arises from a mutation in the lysosomal efflux pump, cystinosin (or …

Cellular redox status affects diverse cellular functions, including proliferation, protein
homeostasis, and aging. Thus, individual differences in redox status can give rise to distinct …

We have previously established that mitochondrial Complex I (CI) mutants of Candida
albicans display reduced oxygen consumption, decreased ATP production, and increased …

The transposition of insertion sequence elements was evaluated among different
Deinococcus geothermalis lineages, including the wild-type, a cystine importer-disrupted …

Abstract Type 4 P-type ATPases (P4-ATPases) function as phospholipid flippases, which
translocate phospholipids from the exoplasmic leaflet to the cytoplasmic leaflet of the lipid …

Lysosomal storage diseases (LSDs) arise from monogenic deficiencies in lysosomal
proteins and pathways and are characterized by a tissue-wide accumulation of a vast variety …