Differently increased volumes of multiple brain areas in
2-hydroxypropyl-β-cyclodextrin
NPC1
brain areas
fresh volumes
lipid storage disorder
miglustat
mouse
treatment
Journal
Frontiers in neuroanatomy
ISSN: 1662-5129
Titre abrégé: Front Neuroanat
Pays: Switzerland
ID NLM: 101477943
Informations de publication
Date de publication:
2024
2024
Historique:
received:
10
05
2024
accepted:
01
07
2024
medline:
31
7
2024
pubmed:
31
7
2024
entrez:
31
7
2024
Statut:
epublish
Résumé
Niemann-Pick disease type C1 (NPC1, MIM 257220) is a heritable lysosomal storage disease characterized by a progressive neurological degeneration that causes disability and premature death. A murine model of In order to evaluate alterations of different brain areas caused by pharmacotherapy, fresh volumes (volumes calculated from the volumes determined from paraffin embedded brain slices) of various brain structures in sham- and drug-treated wild type and mutant mice were measured using stereological methods. In the wild type mice, the volumes of investigated brain areas were not significantly altered by either therapy. Compared with the respective wild types, fresh volumes of specific brain areas, which were significantly reduced in sham-treated Volumes of brain areas of
Sections du résumé
Background
UNASSIGNED
Niemann-Pick disease type C1 (NPC1, MIM 257220) is a heritable lysosomal storage disease characterized by a progressive neurological degeneration that causes disability and premature death. A murine model of
Methods
UNASSIGNED
In order to evaluate alterations of different brain areas caused by pharmacotherapy, fresh volumes (volumes calculated from the volumes determined from paraffin embedded brain slices) of various brain structures in sham- and drug-treated wild type and mutant mice were measured using stereological methods.
Results
UNASSIGNED
In the wild type mice, the volumes of investigated brain areas were not significantly altered by either therapy. Compared with the respective wild types, fresh volumes of specific brain areas, which were significantly reduced in sham-treated
Discussion
UNASSIGNED
Volumes of brain areas of
Identifiants
pubmed: 39081805
doi: 10.3389/fnana.2024.1430790
pmc: PMC11286580
doi:
Types de publication
Journal Article
Langues
eng
Pagination
1430790Informations de copyright
Copyright © 2024 Antipova, Heimes, Seidel, Schulz, Schmitt, Holzmann, Rolfs, Bidmon, González de San Román Martín, Huesgen, Amunts, Keiler, Hammer, Witt and Wree.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.