Interaction between galectin-3 and cystinosin uncovers a pathogenic role of inflammation in kidney involvement of cystinosis.
Amino Acid Transport Systems, Neutral
/ genetics
Animals
Chemokine CCL2
/ immunology
Cystine
/ metabolism
Cystinosis
/ complications
Disease Models, Animal
Disease Progression
Fanconi Syndrome
/ immunology
Female
Galectin 3
/ genetics
Humans
Inflammation
/ immunology
Kidney Tubules, Proximal
/ immunology
Lysosomes
/ metabolism
Macrophages
/ immunology
Male
Mice
Mice, Knockout
Monocytes
/ immunology
Proteolysis
chronic kidney disease
cystinosis
galectin-3
inflammation
monocyte chemoattractant protein–1
Journal
Kidney international
ISSN: 1523-1755
Titre abrégé: Kidney Int
Pays: United States
ID NLM: 0323470
Informations de publication
Date de publication:
08 2019
08 2019
Historique:
received:
18
07
2018
revised:
11
12
2018
accepted:
10
01
2019
pubmed:
1
4
2019
medline:
2
10
2020
entrez:
1
4
2019
Statut:
ppublish
Résumé
Inflammation is involved in the pathogenesis of many disorders. However, the underlying mechanisms are often unknown. Here, we test whether cystinosin, the protein involved in cystinosis, is a critical regulator of galectin-3, a member of the β-galactosidase binding protein family, during inflammation. Cystinosis is a lysosomal storage disorder and, despite ubiquitous expression of cystinosin, the kidney is the primary organ impacted by the disease. Cystinosin was found to enhance lysosomal localization and degradation of galectin-3. In Ctns
Identifiants
pubmed: 30928021
pii: S0085-2538(19)30172-3
doi: 10.1016/j.kint.2019.01.029
pmc: PMC7269416
mid: NIHMS1585184
pii:
doi:
Substances chimiques
Amino Acid Transport Systems, Neutral
0
Ccl2 protein, mouse
0
Chemokine CCL2
0
Galectin 3
0
Lgals3 protein, mouse
0
cystinosin protein, mouse
0
Cystine
48TCX9A1VT
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
350-362Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL088256
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK110162
Pays : United States
Organisme : NINDS NIH HHS
ID : P30 NS047101
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK090058
Pays : United States
Organisme : NEI NIH HHS
ID : R21 EY028642
Pays : United States
Commentaires et corrections
Type : CommentIn
Informations de copyright
Copyright © 2019 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.
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