Elevated granulocyte-colony stimulating factor and hematopoietic stem cell mobilization in Niemann-Pick type C1 disease.

Hematopoietic Stem Cell Mobilization
animal models cholesterol/trafficking hematopoietic stem cells inflammation macrophages/monocytes neutrophils splenomegaly storage diseases

Journal

Journal of lipid research
ISSN: 1539-7262
Titre abrégé: J Lipid Res
Pays: United States
ID NLM: 0376606

Informations de publication

Date de publication:
02 2022
Historique:
received: 10 09 2021
revised: 17 12 2021
accepted: 19 12 2021
pubmed: 11 1 2022
medline: 24 3 2022
entrez: 10 1 2022
Statut: ppublish

Résumé

Niemann-Pick type C1 (NPC1) disease is a progressive lysosomal storage disorder caused by mutations of the NPC1 gene. While neurodegeneration is the most severe symptom, a large proportion of NPC1 patients also present with splenomegaly, which has been attributed to cholesterol and glycosphingolipid accumulation in late endosomes and lysosomes. However, recent data also reveal an increase in the inflammatory monocyte subset in the Npc1

Identifiants

DOI: 10.1016/j.jlr.2021.100167 PMID: 35007562 PMC: PMC8953690
pubmed: 35007562
pii: S0022-2275(21)00150-4
doi: 10.1016/j.jlr.2021.100167
pmc: PMC8953690
pii:
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

100167

Informations de copyright

Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.

Déclaration de conflit d'intérêts

Conflict of interest The authors declare that they have no conflicts of interest with the contents of this article.

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Auteurs

Anouk G Groenen (AG)

Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Anouk M La Rose (AM)

Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Mengying Li (M)

Department of Genetics and Cell Biology, School of Nutrition and Translational Research in Metabolism (NUTRIM), University of Maastricht, Maastricht, The Netherlands.

Venetia Bazioti (V)

Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Arthur F Svendsen (AF)

European Research Institute for the Biology of Ageing, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Niels J Kloosterhuis (NJ)

Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Albertina Ausema (A)

European Research Institute for the Biology of Ageing, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Alle Pranger (A)

Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

M Rebecca Heiner-Fokkema (MR)

Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Klary E Niezen-Koning (KE)

Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Tom Houben (T)

Department of Genetics and Cell Biology, School of Nutrition and Translational Research in Metabolism (NUTRIM), University of Maastricht, Maastricht, The Netherlands.

Ronit Shiri-Sverdlov (R)

Department of Genetics and Cell Biology, School of Nutrition and Translational Research in Metabolism (NUTRIM), University of Maastricht, Maastricht, The Netherlands.

Marit Westerterp (M)

Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Electronic address: m.westerterp@umcg.nl.

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Classifications MeSH

questionsmedicales.fr › Thérapeutique › Biothérapie › Mobilisation de cellules souches hématopoïétiques › Elevated granulocyte-colony stimulating factor...