ArticleNeurobiology of disease2010
Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination.
Article in Neurobiology of disease, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 40 citations in OpenAlex.
- Targeting enhancing myelin regeneration reverses cognitive deficits in a mouse model of intellectual disability.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2026Article
- Dysregulated lipid metabolism and hypomyelination in postnatal peroxisome-deficientFrontiers in molecular neuroscience · 2026Article
- Central Dysmyelination in SSADH-Deficient Humans and Mice.Annals of clinical and translational neurology · 2025Observational
- Enhancing Spatial Transcriptomics via Spatially Constrained Matrix Decomposition with EDGES.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- Aspartate in the Brain: A Review.Neurochemical research · 2025Review
- Myelin lesion in the aspartoacylase (Aspa) knockout rat, an animal model for Canavan disease.Experimental animals · 2024Article
- Deep mutational scanning reveals a correlation between degradation and toxicity of thousands of aspartoacylase variants.Nature communications · 2024Article
- Evidence That DDR1 Promotes Oligodendrocyte Differentiation during Development and Myelin Repair after Injury.International journal of molecular sciences · 2023Article
- ADAMTS4 Enhances Oligodendrocyte Differentiation and Remyelination by Cleaving NG2 Proteoglycan and AttenuatingThe Journal of neuroscience : the official journal of the Society for Neuroscience · 2023Article
- Prognostic significance of carboxypeptidase Q and its methylation in glioblastoma.Translational cancer research · 2023Article
- Acetate Revisited: A Key Biomolecule at the Nexus of Metabolism, Epigenetics, and Oncogenesis - Part 2: Acetate and ACSS2 in Health and Disease.Frontiers in physiology · 2020Review
- Intellectual and developmental disabilities research centers: Fifty years of scientific accomplishments.Annals of neurology · 2019Review
- Behavioral impairment in SHATI/NAT8L knockout mice via dysfunction of myelination development.Scientific reports · 2017Article
- The neuronal metabolite NAA regulates histone H3 methylation in oligodendrocytes and myelin lipid composition.Experimental brain research · 2017Article
- Knockdown of Unconventional Myosin ID Expression Induced Morphological Change in Oligodendrocytes.ASN neuro · 2016Article
- Myosin Id is required for planar cell polarity in ciliated tracheal and ependymal epithelial cells.Cytoskeleton (Hoboken, N.J.) · 2015Article
- Short-term cuprizone feeding verifies N-acetylaspartate quantification as a marker of neurodegeneration.Journal of molecular neuroscience : MN · 2015Article
- Voltage-gated Ca2+ entry promotes oligodendrocyte progenitor cell maturation and myelination in vitro.Experimental neurology · 2015Article
- The adhesion GPCR Gpr56 regulates oligodendrocyte development via interactions with Gα12/13 and RhoA.Nature communications · 2015Article
- N-acetylaspartate (NAA) and N-acetylaspartylglutamate (NAAG) promote growth and inhibit differentiation of glioma stem-like cells.The Journal of biological chemistry · 2013Article
Corrections and comments
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Authors and funding
7 authors at 4 institutions in 1 country.
Funding
Abstract
Canavan disease (CD) is a neurodegenerative disease, caused by a deficiency in the enzyme aspartoacylase (ASPA). This enzyme has been localized to oligodendrocytes; however, it is still undefined how ASPA deficiency affects oligodendrocyte development. In normal mice the pattern of ASPA expression coincides with oligodendrocyte maturation. Therefore, postnatal oligodendrocyte maturation was analyzed in ASPA-deficient mice (CD mice). Early in development, CD mice brains showed decreased expression of neural cell markers that was later compensated. In addition, the levels of myelin proteins were decreased along with abnormal myelination in CD mice compared to wild-type (WT). These defects were associated with increased global levels of acetylated histone H3, decreased chromatin compaction and increased GFAP protein, a marker for astrogliosis. Together, these findings strongly suggest that, early in postnatal development, ASPA deficiency affects oligodendrocyte maturation and myelination.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.