Soft X-ray spectromicroscopy of human fibroblasts with impaired sialin function.


Journal

RSC advances
ISSN: 2046-2069
Titre abrégé: RSC Adv
Pays: England
ID NLM: 101581657

Informations de publication

Date de publication:
04 Sep 2024
Historique:
received: 30 07 2024
accepted: 27 08 2024
medline: 11 9 2024
pubmed: 11 9 2024
entrez: 11 9 2024
Statut: epublish

Résumé

Salla disease (SD) is a lysosomal storage disease where free sialic acid (SA) accumulates in lysosomes due to the impaired function of a membrane protein, sialin. Synchrotron radiation-based scanning transmission soft X-ray spectromicroscopy (STXM) was used to analyze both SD patients' fibroblasts and normal human dermal fibroblasts (NHDF) from healthy controls. Both cell lines were also cultured with

Identifiants

pubmed: 39257666
doi: 10.1039/d4ra05520a
pii: d4ra05520a
pmc: PMC11385984
doi:

Types de publication

Journal Article

Langues

eng

Pagination

28797-28806

Informations de copyright

This journal is © The Royal Society of Chemistry.

Déclaration de conflit d'intérêts

There are no conflicts to declare.

Auteurs

Tuomas Mansikkala (T)

Nano and Molecular Systems Research Unit, 90014 University of Oulu PO Box 3000 Finland leo.mansikkala@oulu.fi minna.patanen@oulu.fi.
Biocenter Oulu, 90014 University of Oulu PO Box 5000 Finland.

Salla M Kangas (SM)

Research Unit of Clinical Medicine and Medical Research Center, Oulu University Hospital and University of Oulu 90014 Oulu Finland.

Ilkka Miinalainen (I)

Biocenter Oulu, 90014 University of Oulu PO Box 5000 Finland.

Pia Angervaniva (P)

Research Unit of Clinical Medicine and Medical Research Center, Oulu University Hospital and University of Oulu 90014 Oulu Finland.

Niklas Darin (N)

Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg 40530 Gothenburg Sweden.

Maria Blomqvist (M)

Department of Laboratory Medicine, Institute of Biomedicine, University of Gothenburg, Sweden and Department of Clinical Chemistry, Sahlgrenska University Hospital 41345 Gothenburg Sweden.

Reetta Hinttala (R)

Biocenter Oulu, 90014 University of Oulu PO Box 5000 Finland.
Research Unit of Clinical Medicine and Medical Research Center, Oulu University Hospital and University of Oulu 90014 Oulu Finland.

Marko Huttula (M)

Nano and Molecular Systems Research Unit, 90014 University of Oulu PO Box 3000 Finland leo.mansikkala@oulu.fi minna.patanen@oulu.fi.

Johanna Uusimaa (J)

Research Unit of Clinical Medicine and Medical Research Center, Oulu University Hospital and University of Oulu 90014 Oulu Finland.
Department of Paediatrics and Adolescent Medicine, Paediatric Neurology Unit, Oulu University Hospital 90029 Oulu Finland.

Minna Patanen (M)

Nano and Molecular Systems Research Unit, 90014 University of Oulu PO Box 3000 Finland leo.mansikkala@oulu.fi minna.patanen@oulu.fi.
Biocenter Oulu, 90014 University of Oulu PO Box 5000 Finland.

Classifications MeSH