Publication:
Bone marrow transplantation improves proximal tubule dysfunction in a mouse model of Dent disease

Date

Date

Date
2017
Journal Article
Published version

Abstract

Abstract

Abstract
Dent disease is a rare X-linked tubulopathy caused by mutations in the endosomal chloride-proton exchanger (ClC-5) resulting in defective receptor-mediated endocytosis and severe proximal tubule dysfunction. Bone marrow transplantation has recently been shown to preserve kidney function in cystinosis, a lysosomal storage disease causing proximal tubule dysfunction. Here we test the effects of bone marrow transplantation in Clcn5(Y/-) mice, a faithful model for Dent disease. Transplantation of wild-type bone marrow in Clcn5(Y/-) mice s

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112 since deposited on 2017-04-27
111last week
Acq. date: 2025-11-12

Additional indexing

Creators (Authors)

  • Gabriel, Sarah S
    affiliation.icon.alt
  • Belge, Hendrica
    affiliation.icon.alt
  • Gassama, Alkaly
    affiliation.icon.alt
  • Debaix, Huguette
    affiliation.icon.alt
  • Luciani, Alessandro
    affiliation.icon.alt
  • Fehr, Thomas
    affiliation.icon.alt
  • Devuyst, Olivier
    affiliation.icon.alt

Journal/Series Title

Journal/Series Title

Journal/Series Title

Volume

Volume

Volume
91

Number

Number

Number
4

Page range/Item number

Page range/Item number

Page range/Item number
842

Page end

Page end

Page end
855

Item Type

Item Type

Item Type
Journal Article

Dewey Decimal Classifikation

Dewey Decimal Classifikation

Dewey Decimal Classifikation

Language

Language

Language
English

Publication date

Publication date

Publication date
2017-04

Date available

Date available

Date available
2017-04-27

Publisher

Publisher

Publisher

ISSN or e-ISSN

ISSN or e-ISSN

ISSN or e-ISSN
0085-2538

OA Status

OA Status

OA Status
Closed

Free Access at

Free Access at

Free Access at
DOI

PubMed ID

PubMed ID

PubMed ID

Metrics

Views

112 since deposited on 2017-04-27
111last week
Acq. date: 2025-11-12
Closed
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