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Botulinum neurotoxin type-A (BoNT/A), which blocks quantal acetylcholine (ACh) release at the neuromuscular junction (NMJ), has demonstrated its efficacy in the symptomatic treatment of blepharospasm. In 3.89% of patients treated for blepharospasm at Tenon Hospital, BoNT/A was no longer effective in relieving the patient's symptoms, and a partial upper myectomy of the muscle was performed.

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Objectives: Recently, our laboratory has discovered a self-innervating population of muscle cells, called motor endplate-expressing cells (MEEs). The cells innately release a wide variety of neurotrophic factors into the microenvironment promoting innervation when used as an injectable treatment. Unlike other stem cells, the therapeutic potential of MEEs is dependent on the cells' ability to maintain phenotypical cell surface proteins in particular motor endplates (MEPs).

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[Peripheral neuroimmunological diseases - Neuropathological insights and clinical perspectives].

Nervenarzt

October 2024

Institut für Neuropathologie, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, und Berlin Institute of Health (BIH), Charité-Universitätsmedizin, Berlin, Deutschland.

Article Synopsis
  • - The article focuses on peripheral neuroimmunological diseases, discussing the varying mechanisms, manifestations, and treatment advancements for conditions like idiopathic inflammatory myopathies and chronic inflammatory demyelinating polyneuropathy.
  • - It emphasizes the importance of muscle biopsies for accurate diagnoses, particularly in distinguishing between vasculitic myopathy and myositis, as well as identifying specific autoimmune characteristics through autoantibody testing.
  • - The text highlights that ongoing research and clinical trials aim to enhance understanding and treatment strategies for these diverse diseases, potentially leading to significant advancements in patient care.
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Analysis of the neuromuscular deficits caused by STAM1 deficiency.

Curr Res Neurobiol

August 2024

Department of Neurobiology, Evelyn F. McKnight Brain Institute, Civitan International Research Center, University of Alabama at Birmingham, Birmingham, 35294, Alabama, USA.

The endosomal sorting complexes required for transport (ESCRT) pathway is composed of a series of protein complexes that are essential for sorting cargo through the endosome. In neurons, the ESCRT pathway is a key mediator of many cellular pathways that regulate neuronal morphogenesis as well as synaptic growth and function. The ESCRT-0 complex, consisting of HGS (hepatocyte growth factor-regulated tyrosine kinase substrate) and STAM (signal-transducing adaptor molecule), acts as a gate keeper to this pathway, ultimately determining the fate of the endosomal cargo.

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Background: Considering the complex pathological mechanisms behind spinal cord injury (SCI) and the adverse effects of present non-approved drugs against SCI, new studies are needed to introduce novel multi-target active ingredients with higher efficacy and lower side effects. Polydatin (PLD) is a naturally occurring stilbenoid glucoside recognized for its antioxidative and anti-inflammatory properties. This study aimed to assess the effects of PLD on sensory-motor function following SCI in rats.

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