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000278040 041__ $$aEnglish
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000278040 1001_ $$0P:(DE-2719)9002065$$aYuan, Xidi$$b0$$eFirst author$$udzne
000278040 245__ $$aTherapeutic sphingosine-1-phosphate receptor modulation by repurposing fingolimod (FTY720) leads to mitigated neuropathy and improved clinical outcome in a mouse model for Charcot-Marie-Tooth 1X disease.
000278040 260__ $$aAmsterdam [u.a.]$$bElsevier Science$$c2025
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000278040 520__ $$aPrevious studies have shown that both the innate and adaptive immune systems foster progression of neuropathy and clinical symptoms in a mouse model for Charcot-Marie-Tooth 1X disease. Here we demonstrate a possible therapeutic translation of these findings using the clinically approved sphingosine-1-phosphate receptor modulator fingolimod (FTY720) in connexin32-deficient mice mimicking Charcot-Marie-Tooth 1X disease. Treatment with FTY720 prevented an increase of CD8+ and CD4+ T-lymphocyte numbers in both femoral quadriceps nerve as well as in ventral spinal roots. While macrophages of ventral spinal roots show a similar, albeit non-significant trend, macrophages from quadriceps nerve are not reduced upon treatment. On the histopathological level, axonopathic changes were reduced in ventral spinal roots, but not in quadriceps nerves upon treatment. Electrophysiological recordings displayed improved nerve conduction parameters upon FTY720 treatment, while clinically, FTY720 treatment ameliorated distinct parameters of motor performance and grip strength. We suggest that targeting the adaptive immune system might be a pharmacological treatment option for mitigating disease burden particularly in severe cases of Charcot-Marie-Tooth 1X.
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000278040 650_7 $$2Other$$aAxon damage
000278040 650_7 $$2Other$$aFTY720
000278040 650_7 $$2Other$$aFingolimod
000278040 650_7 $$2Other$$aInherited peripheral neuropathy
000278040 650_7 $$2Other$$aMacrophages
000278040 650_7 $$2Other$$aMyelin
000278040 650_7 $$2Other$$aNeuroinflammation
000278040 650_7 $$2Other$$aT-lymphocytes
000278040 650_2 $$2MeSH$$aAnimals
000278040 650_2 $$2MeSH$$aFingolimod Hydrochloride: pharmacology
000278040 650_2 $$2MeSH$$aFingolimod Hydrochloride: therapeutic use
000278040 650_2 $$2MeSH$$aCharcot-Marie-Tooth Disease: drug therapy
000278040 650_2 $$2MeSH$$aCharcot-Marie-Tooth Disease: physiopathology
000278040 650_2 $$2MeSH$$aCharcot-Marie-Tooth Disease: pathology
000278040 650_2 $$2MeSH$$aCharcot-Marie-Tooth Disease: immunology
000278040 650_2 $$2MeSH$$aMice
000278040 650_2 $$2MeSH$$aDisease Models, Animal
000278040 650_2 $$2MeSH$$aSphingosine 1 Phosphate Receptor Modulators: pharmacology
000278040 650_2 $$2MeSH$$aSphingosine 1 Phosphate Receptor Modulators: therapeutic use
000278040 650_2 $$2MeSH$$aNeural Conduction: drug effects
000278040 650_2 $$2MeSH$$aSphingosine-1-Phosphate Receptors
000278040 650_2 $$2MeSH$$aGap Junction beta-1 Protein
000278040 650_2 $$2MeSH$$aDrug Repositioning
000278040 650_2 $$2MeSH$$aSpinal Nerve Roots: drug effects
000278040 650_2 $$2MeSH$$aSpinal Nerve Roots: pathology
000278040 650_2 $$2MeSH$$aImmunosuppressive Agents: pharmacology
000278040 650_2 $$2MeSH$$aImmunosuppressive Agents: therapeutic use
000278040 650_2 $$2MeSH$$aFemoral Nerve: drug effects
000278040 650_2 $$2MeSH$$aFemoral Nerve: pathology
000278040 650_2 $$2MeSH$$aMacrophages: drug effects
000278040 650_2 $$2MeSH$$aMale
000278040 7001_ $$aKlein, Dennis$$b1
000278040 7001_ $$aMaier, Anna-Maria$$b2
000278040 7001_ $$aMartini, Rudolf$$b3
000278040 773__ $$0PERI:(DE-600)2008287-3$$a10.1016/j.nmd.2025.105345$$gVol. 50, p. 105345 -$$p105345$$tNeuromuscular disorders$$v50$$x0960-8966$$y2025
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