Mass Spectrometry Analysis of Globotriaosylsphingosine and Its Analogues in Dried Blood Spots.

Capitainer®B Fabry disease biomarkers dried blood spots globotriaosylsphingosine lyso-Gb3 analogues mass spectrometry

Journal

International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791

Informations de publication

Date de publication:
06 Feb 2023
Historique:
received: 28 11 2022
revised: 19 01 2023
accepted: 26 01 2023
entrez: 25 2 2023
pubmed: 26 2 2023
medline: 3 3 2023
Statut: epublish

Résumé

Fabry disease (FD) is an X-linked lysosomal storage disorder where impaired α-galactosidase A enzyme activity leads to the intracellular accumulation of undegraded glycosphingolipids, including globotriaosylsphingosine (lyso-Gb

Identifiants

pubmed: 36834643
pii: ijms24043223
doi: 10.3390/ijms24043223
pmc: PMC9966246
pii:
doi:

Substances chimiques

Glycolipids 0
Sphingolipids 0
alpha-Galactosidase EC 3.2.1.22
Biomarkers 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Funds from C. Auray-Blais
ID : None
Organisme : Sanofi Genzyme
ID : None

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Auteurs

Michel Boutin (M)

Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Centre de Recherche-CIUSSS de l'Estrie-CHUS, Université de Sherbrooke, Sherbrooke, QC J1H 5N4, Canada.

Pamela Lavoie (P)

Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Centre de Recherche-CIUSSS de l'Estrie-CHUS, Université de Sherbrooke, Sherbrooke, QC J1H 5N4, Canada.

Margot Beaudon (M)

Institut de Pharmacologie, Université de Sherbrooke, Sherbrooke, QC J1H 5N4, Canada.

Georges Kabala Ntumba (G)

Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Centre de Recherche-CIUSSS de l'Estrie-CHUS, Université de Sherbrooke, Sherbrooke, QC J1H 5N4, Canada.

Daniel G Bichet (DG)

Research Center, Hôpital du Sacré-Coeur de Montreal, University of Montreal and Nephrology Service, Montreal, QC H4J 1C5, Canada.

Bruno Maranda (B)

Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Centre de Recherche-CIUSSS de l'Estrie-CHUS, Université de Sherbrooke, Sherbrooke, QC J1H 5N4, Canada.

Christiane Auray-Blais (C)

Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Centre de Recherche-CIUSSS de l'Estrie-CHUS, Université de Sherbrooke, Sherbrooke, QC J1H 5N4, Canada.

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Classifications MeSH