Early evidence of delayed oligodendrocyte maturation in the mouse model of mucolipidosis type IV.

Age Factors Animals Brain / metabolism Cell Differentiation Cerebellum / metabolism Cerebral Cortex / metabolism Disease Models, Animal Gene Expression Regulation, Developmental Mice, Inbred C57BL Mice, Knockout Mucolipidoses / genetics Myelin Basic Protein / metabolism Myelin Proteins / metabolism Myelin-Associated Glycoprotein / metabolism Oligodendrocyte Precursor Cells / metabolism Oligodendroglia / metabolism Prosencephalon / metabolism Transient Receptor Potential Channels / genetics
Lysosome Mucolipidosis type IV Mucolipin-1 Myelination Oligodendrocyte TRPML1

Journal

Disease models & mechanisms
ISSN: 1754-8411
Titre abrégé: Dis Model Mech
Pays: England
ID NLM: 101483332

Informations de publication

Date de publication:
30 07 2020
Historique:
received: 27 01 2020
accepted: 16 06 2020
pubmed: 27 6 2020
medline: 24 8 2021
entrez: 27 6 2020
Statut: epublish

Résumé

Mucolipidosis type IV (MLIV) is a lysosomal disease caused by mutations in the

Identifiants

DOI: 10.1242/dmm.044230 PMID: 32586947 PMC: PMC7406328
pubmed: 32586947
pii: dmm.044230
doi: 10.1242/dmm.044230
pmc: PMC7406328
pii:
doi:

Substances chimiques

Mag protein, mouse 0
Mbp protein, mouse 0
Mcoln1 protein, mouse 0
Mobp protein, mouse 0
Myelin Basic Protein 0
Myelin Proteins 0
Myelin-Associated Glycoprotein 0
Transient Receptor Potential Channels 0

Types de publication

Comparative Study Journal Article Research Support, N.I.H., Extramural

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : NINDS NIH HHS
ID : R01 NS096755
Pays : United States

Informations de copyright

© 2020. Published by The Company of Biologists Ltd.

Déclaration de conflit d'intérêts

Competing interestsThe authors declare no competing or financial interests.

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Auteurs

Molly Mepyans (M)

Center for Genomic Medicine and Department of Neurology, Massachusetts General Hospital Research Institute and Harvard Medical School, Boston, MA 02114, USA.

Livia Andrzejczuk (L)

Department of Biological Sciences, University of Pittsburgh, Pittsburgh, PA 15260, USA.

Jahree Sosa (J)

Department of Biological Sciences, University of Pittsburgh, Pittsburgh, PA 15260, USA.

Sierra Smith (S)

Center for Genomic Medicine and Department of Neurology, Massachusetts General Hospital Research Institute and Harvard Medical School, Boston, MA 02114, USA.

Shawn Herron (S)

Center for Genomic Medicine and Department of Neurology, Massachusetts General Hospital Research Institute and Harvard Medical School, Boston, MA 02114, USA.

Samantha DeRosa (S)

Center for Genomic Medicine and Department of Neurology, Massachusetts General Hospital Research Institute and Harvard Medical School, Boston, MA 02114, USA.

Susan A Slaugenhaupt (SA)

Center for Genomic Medicine and Department of Neurology, Massachusetts General Hospital Research Institute and Harvard Medical School, Boston, MA 02114, USA.

Albert Misko (A)

Center for Genomic Medicine and Department of Neurology, Massachusetts General Hospital Research Institute and Harvard Medical School, Boston, MA 02114, USA.

Yulia Grishchuk (Y)

Center for Genomic Medicine and Department of Neurology, Massachusetts General Hospital Research Institute and Harvard Medical School, Boston, MA 02114, USA ygrishchuk@partners.org kiselyov@pitt.edu.
ORCID: 0000-0002-4684-4159

Kirill Kiselyov (K)

Department of Biological Sciences, University of Pittsburgh, Pittsburgh, PA 15260, USA ygrishchuk@partners.org kiselyov@pitt.edu.
ORCID: 0000-0001-6683-2895

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Classifications MeSH

questionsmedicales.fr › Environnement et santé publique › Santé publique › Facteurs épidémiologiques › Facteurs âges › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Eucaryotes › Animaux › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Phénomènes physiologiques cellulaires › Différenciation cellulaire › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Tronc cérébral › Rhombencéphale › Métencéphale › Cervelet › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Prosencéphale › Télencéphale › Cerveau › Cortex cérébral › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Techniques d'investigation › Modèles théoriques › Modèles biologiques › Modèles animaux de maladie humaine › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Phénomènes génétiques › Régulation de l'expression des gènes › Régulation de l'expression des gènes au cours du développement › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Lignées consanguines de souris › Souris de lignée C57BL › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Souris transgéniques › Souris knockout › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Erreurs innées du métabolisme › Maladies lysosomiales › Maladies neurologiques de surcharge lysosomiale › Mucolipidoses › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines de tissu nerveux › Protéines de la myéline › Protéine basique de la myéline › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines de tissu nerveux › Protéines de la myéline › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines de tissu nerveux › Protéines de la myéline › Glycoprotéine associée à la myéline › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Cellules › Cellules souches › Cellules souches neurales › Précurseurs des oligodendrocytes › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Cellules › Névroglie › Oligodendroglie › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Prosencéphale › Early evidence of delayed oligodendrocyte...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines membranaires › Protéines de transport membranaire › Canaux ioniques › Canaux cationiques TRP › Early evidence of delayed oligodendrocyte...