ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2006
The structural and functional integrity of peripheral nerves depends on the glial-derived signal desert hedgehog.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers.
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Who cites it
42 citing papers in PubMed, 84 citations in OpenAlex.
- Article
- Peripheral glia diversity.Journal of anatomy · 2022Review
- Schwann cell precursors represent a neural crest-like state with biased multipotency.The EMBO journal · 2022Article
- Gli1 Regulates the Postnatal Acquisition of Peripheral Nerve Architecture.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2022Article
- Case Report: Long-term follow-up of desert hedgehog variant caused 46, XY gonadal dysgenesis with multiple complications in a Chinese child.Frontiers in genetics · 2022Article
- Ptch2 is a Potential Regulator of Mesenchymal Stem Cells.Frontiers in physiology · 2022Article
- Schwann cell interactions during the development of the peripheral nervous system.Developmental neurobiology · 2021Review
- Phagocytosis by Peripheral Glia: Importance for Nervous System Functions and Implications in Injury and Disease.Frontiers in cell and developmental biology · 2021Review
- Hedging against Neuropathic Pain: Role of Hedgehog Signaling in Pathological Nerve Healing.International journal of molecular sciences · 2020Review
- DHH pathogenic variants involved in 46,XY disorders of sex development differentially impact protein self-cleavage and structural conformation.Human genetics · 2020Article
- Deletion of CD38 and supplementation of NADScientific reports · 2020Article
- Oltipraz Prevents High Glucose-Induced Oxidative Stress and Apoptosis in RSC96 Cells through the Nrf2/NQO1 Signalling Pathway.BioMed research international · 2020Article
- Functionally Distinctive Ptch Receptors Establish Multimodal Hedgehog Signaling in the Tooth Epithelial Stem Cell Niche.Stem cells (Dayton, Ohio) · 2019Article
- Liver X Receptors and Their Implications in the Physiology and Pathology of the Peripheral Nervous System.International journal of molecular sciences · 2019Review
- Role of Hedgehog Signaling in Vasculature Development, Differentiation, and Maintenance.International journal of molecular sciences · 2019Review
- CD34 defines melanocyte stem cell subpopulations with distinct regenerative properties.PLoS genetics · 2019Article
- Schwann Cell Precursors; Multipotent Glial Cells in Embryonic Nerves.Frontiers in molecular neuroscience · 2019Review
- Fibrosis: a distinguishing feature in the pathology of neural leprosy.Memorias do Instituto Oswaldo Cruz · 2019Article
- Polycomb repression regulates Schwann cell proliferation and axon regeneration after nerve injury.Glia · 2018Article
- Perineurial Glial Plasticity and the Role of TGF-β in the Development of the Blood-Nerve Barrier.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2017Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 3 countries.
Funding
Abstract
We show that desert hedgehog (dhh), a signaling molecule expressed by Schwann cells, is essential for the structural and functional integrity of the peripheral nerve. Dhh-null nerves display multiple abnormalities that affect myelinating and nonmyelinating Schwann cells, axons, and vasculature and immune cells. Myelinated fibers of these mice have a significantly increased (more than two times) number of Schmidt-Lanterman incisures (SLIs), and connexin 29, a molecular component of SLIs, is strongly upregulated. Crossing Dhh-null mice with myelin basic protein (MBP)-deficient shiverer mice, which also have increased SLI numbers, results in further increased SLIs, suggesting that Dhh and MBP control SLIs by different mechanisms. Unmyelinated fibers are also affected, containing many fewer axons per Schwann cell in transverse profiles, whereas the total number of unmyelinated axons is reduced by approximately one-third. In Dhh-null mice, the blood-nerve barrier is permeable and neutrophils and macrophage numbers are elevated, even in uninjured nerves. Dhh-null nerves also lack the largest-diameter myelinated fibers, have elevated numbers of degenerating myelinated axons, and contain regenerating fibers. Transected dhh nerves degenerate faster than wild-type controls. This demonstrates that a single identified glial signal, Dhh, plays a critical role in controlling the integrity of peripheral nervous tissue, in line with its critical role in nerve sheath development (Parmantier et al., 1999). The complexity of the defects raises a number of important questions about the Dhh-dependent cell-cell signaling network in peripheral nerves.
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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.