Sensory-specific peripheral nerve pathology in a rat model of Fabry disease
| Autor: | Anthony J. Burand, Tyler B. Waltz, Cheryl L. Stucky, Katelyn E. Sadler |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Pathology
medicine.medical_specialty Short Communication Rat model Neuroscience (miscellaneous) Globotriaosylceramide Nerve fiber Sensory system Neurosciences. Biological psychiatry. Neuropsychiatry Nerve pathology Neuropathic pain chemistry.chemical_compound medicine LAMP1 lysosomal-associated membrane protein 1 TEM transmission electron microscopy LM Light microscopy Fabry disease α-GAL A α-galactosidase A IB4 isolectin B4 business.industry Chronic pain Gb3 globotriaosylceramide medicine.disease Lysosome Peripheral DRG dorsal root ganglia Anesthesiology and Pain Medicine medicine.anatomical_structure chemistry Neurology (clinical) business FD Fabry disease RC321-571 |
| Zdroj: | Neurobiology of Pain, Vol 10, Iss, Pp 100074-(2021) Neurobiology of Pain |
| Popis: | Highlights • Fabry disease rats exhibit peripheral nerve pathology, specifically in sensory nerves. • Fabry disease tibial nerves exhibit different pathology according to anatomical location. • Fabry disease associated lipids and lysosomes accumulate in peripheral axons. Fabry disease (FD) causes life-long pain, the mechanisms of which are unclear. Patients with FD have chronic pain that mirrors symptoms of other painful peripheral neuropathies. However, it is unclear what underlying damage occurs in FD peripheral nerves that may contribute to chronic pain. Here, we characterized myelinated and unmyelinated fiber pathology in peripheral nerves of a rat model of FD. Decreased nerve fiber density and increased nerve fiber pathology were noted in unmyelinated and myelinated fibers from FD rats; both observations were dependent on sampled nerve fiber modality and anatomical location. FD myelinated axons exhibited lipid accumulations that were determined to be the FD-associated lipid globotriaosylceramide (Gb3), and to a lesser extent lysosomes. These findings suggest that axonal Gb3 accumulation may drive peripheral neuron dysfunction and subsequent pain in FD. |
| Databáze: | OpenAIRE |
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