Bioengineered Cystinotic Kidney Tubules Recapitulate a Nephropathic Phenotype.
Amino Acid Transport Systems, Neutral
/ metabolism
Animals
Autophagy
Bioengineering
Biomarkers
/ metabolism
Cell Line
Cystine
/ metabolism
Cystinosis
/ pathology
Epithelial Cells
/ pathology
Fluorescein-5-isothiocyanate
/ metabolism
Humans
Inulin
/ metabolism
Ketoglutaric Acids
/ metabolism
Kidney Tubules, Proximal
/ pathology
Lysosomal-Associated Membrane Protein 1
/ metabolism
Membranes, Artificial
Metabolomics
Phenotype
Principal Component Analysis
TOR Serine-Threonine Kinases
/ metabolism
3-dimensional models
autophagy
hollow fiber membrane
lysosomal storage disease
nephropathic cystinosis
Journal
Cells
ISSN: 2073-4409
Titre abrégé: Cells
Pays: Switzerland
ID NLM: 101600052
Informations de publication
Date de publication:
05 01 2022
05 01 2022
Historique:
received:
25
11
2021
revised:
21
12
2021
accepted:
25
12
2021
entrez:
11
1
2022
pubmed:
12
1
2022
medline:
4
3
2022
Statut:
epublish
Résumé
Nephropathic cystinosis is a rare and severe disease caused by disruptions in the
Identifiants
pubmed: 35011739
pii: cells11010177
doi: 10.3390/cells11010177
pmc: PMC8750898
pii:
doi:
Substances chimiques
Amino Acid Transport Systems, Neutral
0
Biomarkers
0
CTNS protein, human
0
Ketoglutaric Acids
0
Lysosomal-Associated Membrane Protein 1
0
Membranes, Artificial
0
Cystine
48TCX9A1VT
Inulin
9005-80-5
TOR Serine-Threonine Kinases
EC 2.7.11.1
Fluorescein-5-isothiocyanate
I223NX31W9
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Health Holland
ID : LSHM20009
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