Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype
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چکیده
منابع مشابه
Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype
The first symptom arising in many Fabry patients is neuropathic pain due to changes in small myelinated and unmyelinated fibers in the periphery, which is subsequently followed by a loss of sensory perception. Here we studied changes in the peripheral nervous system of Fabry patients and a Fabry mouse model induced by deletion of α-galactosidase A (Gla-/0). The skin innervation of Gla-/0 mice r...
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BACKGROUND AND PURPOSE Dolichoectasia of the basilar artery is a characteristic finding of Fabry disease. However, its prevalence, severity, and course have been poorly studied. This study quantitatively evaluated, by MRA, a panel of basilar artery parameters in a large cohort of patients with Fabry disease. MATERIALS AND METHODS Basilar artery mean diameter, curved length, "origin-to-end" li...
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Fabry disease is an X-chromosome-linked lysosomal storage disease characterized by a deficient activity or, in most males, absence of the enzyme α-galactosidase A (a-Gal A) leading to systemic, primary lysosomal accumulation of globotriaosylceramide (Gb3) (1). Recent literature refers to an overall birth prevalence of 1:40,000-170,000; however, such data do not allow an estimation on an actual ...
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ژورنال
عنوان ژورنال: Frontiers in Neurology
سال: 2017
ISSN: 1664-2295
DOI: 10.3389/fneur.2017.00335