Results 51 to 60 of about 888 (137)

Impacts of hnRNP A1 Splicing Inhibition on the Brain Remyelination Proteome

Journal of Neurochemistry, Volume 169, Issue 1, January 2025.
This study highlights the effects of hnRNP A1 on myelin proteins in the prefrontal cortex, corpus callosum, and hippocampus of adult mice following cuprizone‐induced demyelination and subsequent hnRNP A1 splicing inhibition (VPC‐8001) during remyelination. VPC‐8001 exposure induced significant changes in myelination proteins such as MOG, MAG, MBP, CNP,
Caroline Brandão‐Teles, Victor Corasolla Carregari, Guilherme Reis‐de‐Oliveira, Bradley J. Smith, Yane Chaves, Aline Valéria Sousa Santos, Erick Martins de Carvalho Pinheiro, Caio C. Oliveira, Andre Schwambach Vieira, Fernanda Crunfli, Daniel Martins‐de‐Souza   +10 more
wiley   +1 more source

N-Acetylaspartate Metabolism Outside the Brain: Lipogenesis, Histone Acetylation, and Cancer

Frontiers in Endocrinology, 2017
N-acetylaspartate (NAA) is a highly abundant brain metabolite. Aberrant NAA concentrations have been detected in many pathological conditions and although the function of NAA has been extensively investigated in the brain it is still controversial.
Juliane G. Bogner-Strauss, Juliane G. Bogner-Strauss   +1 more
doaj   +1 more source

Structural modeling of p.V31F variant in the aspartoacylase gene

Metabolic Brain Disease, 2016
Aspartoacylase (ASPA) is an abundant enzyme in the brain, which catalyzes the conversion of N-acetylaspartate into acetate and aspartate, deficiency in its activity leads to degeneration of the white matter of the brain and is a recognized cause of Canavan disease (CD), which affect children.
Krishnamoorthy, Navaneethakrishnan, Zayed, Hatem   +1 more
openaire   +4 more sources

Developmental and regional distribution of aspartoacylase in rat brain tissue [PDF]

Journal of Neurochemistry, 2001
The function of N‐acetyl‐aspartate (NAA), a predominant molecule in the brain, has not yet been determined. However, NAA is commonly used as a putative marker of viable neurones. To investigate the possible function of NAA, we determined the anatomical, developmental and cellular distribution of aspartoacylase, which catalyses the hydrolysis of NAA ...
K K, Bhakoo, T J, Craig, P, Styles
openaire   +2 more sources

Systemic Treatment of Body‐Wide Duchenne Muscular Dystrophy Symptoms

Clinical Pharmacology &Therapeutics, Volume 116, Issue 6, Page 1472-1484, December 2024.
Duchenne muscular dystrophy (DMD) is a fatal X‐linked disease that leads to premature death due to the loss of dystrophin. Current strategies predominantly focus on the therapeutic treatment of affected skeletal muscle tissue. However, certain results point to the fact that with successful treatment of skeletal muscle, DMD‐exposed latent phenotypes in ...
Patryk Konieczny
wiley   +1 more source

Loss of central auditory processing in a mouse model of Canavan disease.

PLoS ONE, 2014
Canavan Disease (CD) is a leukodystrophy caused by homozygous null mutations in the gene encoding aspartoacylase (ASPA). ASPA-deficiency is characterized by severe psychomotor retardation, and excessive levels of the ASPA substrate N-acetylaspartate (NAA)
Georg von Jonquieres, Kristina E Froud, Claudia B Klugmann, Ann C Y Wong, Gary D Housley, Matthias Klugmann   +5 more
doaj   +1 more source

Bimodal occurrence of aspartoacylase in myelin and cytosol of brain [PDF]

Journal of Neurochemistry, 2006
AbstractThe growing use of N‐acetylaspartate as an indicator of neuronal viability has fostered interest in the biological function(s) of this unusual amino acid derivative. In considering the various physiological roles that have been proposed for this relatively abundant molecule one is obliged to take into account its unusual metabolic ...
Jianfeng, Wang, Reuben, Matalon, Gita, Bhatia, Gusheng, Wu, Hong, Li, Tong, Liu, Zi-Hua, Lu, Robert W, Ledeen   +7 more
openaire   +2 more sources

Multiomics analysis reveals the potential mechanism of high‐fat diet in dextran sulfate sodium‐induced colitis mice model

Food Science &Nutrition, Volume 12, Issue 10, Page 8309-8323, October 2024.
HFD‐treated mice exhibited notable alternations in the structure and composition of the gut microbiota, with some of these alternations being associated with the pathogenesis of IBD. Analysis of the colon transcriptome revealed 11 hub genes and 7 hub pathways among control, DSS‐induced colitis, and HFD + DSS‐treated groups. Impact of HFD on DSS‐induced
Yuyang Zhao, Zhimin Chen, Ruiyi Dong, Yufan Liu, Yixin Zhang, Yan Guo, Meiyi Yu, Xiang Li, Jiangbin Wang   +8 more
wiley   +1 more source

Glial promoter selectivity following AAV-delivery to the immature brain.

PLoS ONE, 2013
Recombinant adeno-associated virus (AAV) vectors are versatile tools for gene transfer to the central nervous system (CNS) and proof-of-concept studies in adult rodents have shown that the use of cell type-specific promoters is sufficient to target AAV ...
Georg von Jonquieres, Nadine Mersmann, Claudia Bettina Klugmann, Anne Editha Harasta, Beat Lutz, Orla Teahan, Gary David Housley, Dominik Fröhlich, Eva-Maria Krämer-Albers, Matthias Klugmann   +9 more
doaj   +1 more source

Deficient brain GABA metabolism leads to widespread impairments of astrocyte and oligodendrocyte function

Glia, Volume 72, Issue 10, Page 1821-1839, October 2024.
Main Points Glial dysfunction is intimately linked to SSADH deficiency pathology. Astrocyte metabolism, glutamine synthesis and neurotransmitter recycling are severely perturbed. Oligodendrocyte associated proteins and myelin‐enriched sphingolipids are depleted. Abstract The neurometabolic disorder succinic semialdehyde dehydrogenase (SSADH) deficiency
Jens V. Andersen, Oana C. Marian, Filippa L. Qvist, Emil W. Westi, Blanca I. Aldana, Arne Schousboe, Anthony S. Don, Niels H. Skotte, Petrine Wellendorph   +8 more
wiley   +1 more source