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2015 ; 10
(3
): e0120998
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Endo-lysosomal dysfunction in human proximal tubular epithelial cells deficient
for lysosomal cystine transporter cystinosin
#MMPMID25811383
Ivanova EA
; De Leo MG
; Van Den Heuvel L
; Pastore A
; Dijkman H
; De Matteis MA
; Levtchenko EN
PLoS One
2015[]; 10
(3
): e0120998
PMID25811383
show ga
Nephropathic cystinosis is a lysosomal storage disorder caused by mutations in
the CTNS gene encoding cystine transporter cystinosin that results in
accumulation of amino acid cystine in the lysosomes throughout the body and
especially affects kidneys. Early manifestations of the disease include renal
Fanconi syndrome, a generalized proximal tubular dysfunction. Current therapy of
cystinosis is based on cystine-lowering drug cysteamine that postpones the
disease progression but offers no cure for the Fanconi syndrome. We studied the
mechanisms of impaired reabsorption in human proximal tubular epithelial cells
(PTEC) deficient for cystinosin and investigated the endo-lysosomal compartments
of cystinosin-deficient PTEC by means of light and electron microscopy. We
demonstrate that cystinosin-deficient cells had abnormal shape and distribution
of the endo-lysosomal compartments and impaired endocytosis, with decreased
surface expression of multiligand receptors and delayed lysosomal cargo
processing. Treatment with cysteamine improved surface expression and lysosomal
cargo processing but did not lead to a complete restoration and had no effect on
the abnormal morphology of endo-lysosomal compartments. The obtained results
improve our understanding of the mechanism of proximal tubular dysfunction in
cystinosis and indicate that impaired protein reabsorption can, at least
partially, be explained by abnormal trafficking of endosomal vesicles.
|Amino Acid Transport Systems, Neutral/deficiency/genetics/*metabolism
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