ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2007
Autonomic denervation of lymphoid organs leads to epigenetic immune atrophy in a mouse model of Krabbe disease.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed, 57 citations in OpenAlex.
- The thymus gland is a target and maintainer of amyotrophic lateral sclerosis pathophysiology.Brain, behavior, & immunity - health · 2026Article
- Neural regulation of the thymus: past, current, and future perspectives.Frontiers in immunology · 2025Review
- Review
- Can early treatment of twitcher mice with high dose AAVrh10-GALC eliminate the need for BMT?BioImpacts : BI · 2021Article
- Krabbe Disease: Prospects of Finding a Cure Using AAV Gene Therapy.Frontiers in medicine · 2021Review
- Macrophages Expressing GALC Improve Peripheral Krabbe Disease by a Mechanism Independent of Cross-Correction.Neuron · 2020Article
- β-Galactosylceramidase Deficiency Causes Bone Marrow Vascular Defects in an Animal Model of Krabbe Disease.International journal of molecular sciences · 2019Article
- A HILIC-MS/MS method for simultaneous quantification of the lysosomal disease markers galactosylsphingosine and glucosylsphingosine in mouse serum.Biomedical chromatography : BMC · 2018Article
- Neural pathways involved in infection-induced inflammation: recent insights and clinical implications.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2018Review
- Long-Term Improvement of Neurological Signs and Metabolic Dysfunction in a Mouse Model of Krabbe's Disease after Global Gene Therapy.Molecular therapy : the journal of the American Society of Gene Therapy · 2018Article
- Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease.PloS one · 2017Article
- Mesenchymal Stem Cells Yield Transient Improvements in Motor Function in an Infant Rhesus Macaque with Severe Early-Onset Krabbe Disease.Stem cells translational medicine · 2017Article
- Generation of a LacZ reporter transgenic mouse line for the stereological analysis of oligodendrocyte loss in galactosylceramidase deficiency.Journal of neuroscience research · 2016Article
- Biochemical, cell biological, pathological, and therapeutic aspects of Krabbe's disease.Journal of neuroscience research · 2016Review
- Psychosine inhibits osteoclastogenesis and bone resorption via G protein-coupled receptor 65.Journal of endocrinological investigation · 2015Article
- Combined gene/cell therapies provide long-term and pervasive rescue of multiple pathological symptoms in a murine model of globoid cell leukodystrophy.Human molecular genetics · 2015Article
- Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophy.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2015Article
- Mechanism of neuromuscular dysfunction in Krabbe disease.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2015Article
- Neuronal inclusions of α-synuclein contribute to the pathogenesis of Krabbe disease.The Journal of pathology · 2014Article
- Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture.Journal of lipid research · 2013Article
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Lysosomal beta-galactosylceramidase deficiency results in demyelination and inflammation in the nervous system causing the neurological Krabbe disease. In the Twitcher mouse model of this disease, we found that neurological symptoms parallel progressive and severe lymphopenia. Although lymphopoiesis is normal before disease onset, primary and secondary lymphoid organs progressively degenerate afterward. This occurs despite preserved erythropoiesis and leads to severe peripheral lymphopenia caused by reduced numbers of T cell precursors and mature lymphocytes. Hematopoietic cell replacement experiments support the existence of an epigenetic factor in mutant mice reconcilable with a progressive loss of autonomic axons that hampers thymic functionality. We propose that degeneration of autonomic nerves leads to the irreversible thymic atrophy and loss of immune-competence. Our study describes a new aspect of Krabbe disease, placing patients at risk of immune-related pathologies, and identifies a novel target for therapeutic interventions.
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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.