Pathological Bergmann glia alterations and disrupted calcium dynamics in ataxic Canavan disease mice.

Humans Child Infant Mice Animals Canavan Disease / genetics Calcium Ataxia / pathology Oligodendroglia / metabolism Neurodegenerative Diseases / pathology Aspartic Acid Atrophy / complications
Bergmann glia Canavan disease Purkinje cell astrocyte calcium astrocytes ataxia cerebellum

Journal

Glia
ISSN: 1098-1136
Titre abrégé: Glia
Pays: United States
ID NLM: 8806785

Informations de publication

Date de publication:
12 2023
Historique:
revised: 11 07 2023
received: 20 12 2022
accepted: 28 07 2023
medline: 26 10 2023
pubmed: 23 8 2023
entrez: 23 8 2023
Statut: ppublish

Résumé

Canavan disease (CD) is a recessively inherited pediatric leukodystrophy resulting from inactivating mutations to the oligodendroglial enzyme aspartoacylase (ASPA). ASPA is responsible for hydrolyzing the amino acid derivative N-acetyl-L-aspartate (NAA), and without it, brain NAA concentrations increase by 50% or more. Infants and children with CD present with progressive cognitive and motor delays, cytotoxic edema, astroglial vacuolation, and prominent spongiform brain degeneration. ASPA-deficient CD mice (Aspa

Identifiants

DOI: 10.1002/glia.24454 PMID: 37610133 PMC: PMC10591969
pubmed: 37610133
doi: 10.1002/glia.24454
pmc: PMC10591969
mid: NIHMS1930234
doi:

Substances chimiques

Calcium SY7Q814VUP
Aspartic Acid 30KYC7MIAI

Types de publication

Journal Article Research Support, Non-U.S. Gov't Research Support, N.I.H., Extramural Research Support, U.S. Gov't, Non-P.H.S.

Langues

eng

Sous-ensembles de citation

IM

Pagination

2832-2849

Subventions

Organisme : NIH HHS
ID : UL1 TR001860
Pays : United States
Organisme : NIH HHS
ID : TL1 TR001861
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS123165
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS105886
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR001860
Pays : United States
Organisme : NINDS NIH HHS
ID : R21 NS117386
Pays : United States
Organisme : NIH HHS
ID : R01NS123080
Pays : United States
Organisme : NIH HHS
ID : R21NS125464
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS113859
Pays : United States
Organisme : NIH HHS
ID : R01NS113859
Pays : United States
Organisme : NCATS NIH HHS
ID : TL1 TR001861
Pays : United States
Organisme : NINDS NIH HHS
ID : R21 NS125464
Pays : United States
Organisme : NIH HHS
ID : R21NS117386
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS123080
Pays : United States
Organisme : NIH HHS
ID : R21NS13381
Pays : United States
Organisme : NIH HHS
ID : R01NS105886
Pays : United States
Organisme : NIH HHS
ID : R01NS123165
Pays : United States

Informations de copyright

© 2023 The Authors. GLIA published by Wiley Periodicals LLC.

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Auteurs

Vanessa L Hull (VL)

Department of Neurology, University of California Davis School of Medicine, Sacramento, California, USA.
Institute for Pediatric Regenerative Medicine, Shriners Hospital for Children, Sacramento, California, USA.
ORCID: 0000-0003-3221-740X

Yan Wang (Y)

Department of Neurology, University of California Davis School of Medicine, Sacramento, California, USA.
Institute for Pediatric Regenerative Medicine, Shriners Hospital for Children, Sacramento, California, USA.
ORCID: 0000-0002-7744-4078

Travis Burns (T)

Department of Neurology, University of California Davis School of Medicine, Sacramento, California, USA.
Institute for Pediatric Regenerative Medicine, Shriners Hospital for Children, Sacramento, California, USA.

Sarah Sternbach (S)

Department of Biological Sciences, Kent State University, Kent, Ohio, USA.
ORCID: 0000-0002-5761-9512

Shuaishuai Gong (S)

Department of Neurology, University of California Davis School of Medicine, Sacramento, California, USA.
Institute for Pediatric Regenerative Medicine, Shriners Hospital for Children, Sacramento, California, USA.

Jennifer McDonough (J)

Department of Biological Sciences, Kent State University, Kent, Ohio, USA.
ORCID: 0000-0001-9809-2289

Fuzheng Guo (F)

Department of Neurology, University of California Davis School of Medicine, Sacramento, California, USA.
Institute for Pediatric Regenerative Medicine, Shriners Hospital for Children, Sacramento, California, USA.
ORCID: 0000-0003-3410-8389

Laura N Borodinsky (LN)

Institute for Pediatric Regenerative Medicine, Shriners Hospital for Children, Sacramento, California, USA.
Department of Physiology & Membrane Biology, University of California Davis School of Medicine, Sacramento, California, USA.
ORCID: 0000-0003-2937-7023

David Pleasure (D)

Department of Neurology, University of California Davis School of Medicine, Sacramento, California, USA.
Institute for Pediatric Regenerative Medicine, Shriners Hospital for Children, Sacramento, California, USA.
ORCID: 0000-0002-9122-1268

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questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Pathological Bergmann glia alterations and...
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questionsmedicales.fr › Eucaryotes › Animaux › Pathological Bergmann glia alterations and...
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