163 results match your criteria: "U1195 Inserm-Université Paris Saclay[Affiliation]"

The temporal control of mitotic exit of individual Schwann cells (SCs) is essential for radial sorting and peripheral myelination. However, it remains unknown when, during their multiple rounds of division, SCs initiate myelin signaling in vivo. By manipulating SC division during development, we report that when SCs skip their division during migration, but not during radial sorting, they fail to myelinate peripheral axons.

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Structural remodeling of the brain cortex and functional recovery following hypoglossal-facial neurorrhaphy in patients with facial paralysis.

Brain Res

December 2024

Beijing Key Laboratory of Central Nervous System Injury, Beijing Neurosurgical Institute, Capital Medical University, Beijing 10070, China; Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 10070, China; U1195, Inserm et Universite Paris-Saclay, 94276 Le Kremlin-Bicetre, France. Electronic address:

Objective: Peripheral nerve injury results in functional alterations of the corresponding active brain areas, which are closely related to functional recovery. Whether such functional plasticity induces relative anatomical structural changes remains to be investigated.

Methods: In this study, we investigated the changes in brain cortical thickness in patients with facial paralysis following neurorrhaphy treatment at different follow-up times.

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The walnut tree as a source of progesterone for reproductive control in goats.

Animal

December 2024

Physiologie de la Reproduction et des Comportements, CNRS, INRAE, Université de Tours, 37380 Nouzilly, France.

Intravaginal sponges impregnated with the progesterone (P4) analogue fluorogestone acetate (FGA) induce synchronous oestrous behaviour and normal ovulatory cycle in goats. To explore alternatives using natural P4 from plants, we developed a method of ethanolic extraction and a specific enzyme immunoassay (EIA) to measure P4 in the different parts of the walnut tree Juglans regia. We found a very high concentration of P4, specifically in the leaves of the three most common French varieties (∼100 mg/kg of DM) but not in flowers, fruits, septa, husk, oil or cake.

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The treatment of stress-related disorders such as anxiety and depression is still challenging. One potential therapeutical option are neurosteroids. Their synthesis is promoted by ligands of the mitochondrial translocator protein 18 kDa (TSPO).

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Sex-related differences characterize multiple sclerosis, an autoimmune, inflammatory and neurodegenerative disease displaying higher incidence in females as well as discrepancies in susceptibility and progression. Besides clinical specificities, molecular and cellular differences related to sex hormones were progressively uncovered improving our understanding of the mechanisms involved in this disabling disease. The most recent findings may give rise to the identification of novel therapeutic perspectives that could meet the urgent need for a treatment preventing the transition from the recurrent- to the progressive form of the disease.

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Article Synopsis
  • * Researchers analyzed data from 275 CMTX1 patients across 13 centers in France, finding that those with mutations in transmembrane domains had more severe symptoms and earlier onset than those with mutations in intracellular or extracellular domains.
  • * The findings suggest that the type of genetic mutation not only helps diagnose CMTX1 but also predicts disease severity, emphasizing the need to consider these correlations in upcoming clinical research.
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Progestagens and progesterone receptor modulation: Effects on the brain, mood, stress, and cognition in females.

Front Neuroendocrinol

November 2024

Department of Women's and Children's Health, Science for Life Laboratory, Uppsala University, Sweden. Electronic address:

Article Synopsis
  • - Progesterone, a lipophilic hormone, has significant effects on female behavior and mental health, particularly through its interaction with brain receptors, especially during key life stages like puberty and menopause.
  • - Research indicates that progesterone influences mood regulation, stress response, and cognitive processing, with potential improvements in conditions like premenstrual dysphoric disorder through its modulation of brain activity.
  • - The effects of progestins in contraceptives and hormone therapy on brain function and health outcomes (like stroke protection) are not fully understood, and more studies are needed to clarify how different factors (like dosage and timing) impact these effects.
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Failure of myelin regeneration by oligodendrocytes contributes to progressive decline in many neurological diseases. Here, using in vitro and in vivo rodent models, functional blockade, and mouse brain demyelination, we demonstrate that Sonic hedgehog (Shh) expression in a subset of oligodendrocyte progenitor cells precedes the expression of myelin basic protein (MBP), a major myelin sheath protein. Primary cultures of rodent cortical oligodendrocytes show that Shh mRNA and protein are upregulated during oligodendrocyte maturation before the upregulation of MBP expression.

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Tracking myasthenia gravis severity over time: Insights from the French health insurance claims database.

Eur J Neurol

January 2025

Neurology and Neuromuscular Diseases Department, Neuromuscular Reference Centre AOC, Pellegrin Hospital, Bordeaux University Hospitals, Bordeaux, France.

Background And Purpose: Few data are available on the course of myasthenia gravis (MG) regarding disease severity and stability over time in real-world settings. This study used the French National Health Insurance Database (SNDS) to assess markers of disease severity in patients with MG longitudinally.

Methods: All patients with MG-related claims in the SNDS between 2013 and 2020 were identified.

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Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive Schwann cell-derived sarcomas that are sporadic or associated with Neurofibromatosis 1 (NF1) gene mutations. Traditional therapies are usually ineffective for treating MPNSTs, so new targets need to be identified for the treatment of MPNSTs. In the present study, the role of the mitochondrial translocator protein (TSPO) in the regulation of cell proliferation and the cell cycle in MPNSTs was investigated.

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Background: Timely revascularization in acute arterial ischemic stroke (AIS) is paramount for optimal outcomes. However, factors causing treatment delays in pediatric AIS remain understudied. We investigated determinants affecting the time from symptom onset or last-known-well to the start of recanalization treatment in pediatric AIS.

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Background: Although flow diverters (FDs) have benefited from several technical improvements, recently concerns have arisen regarding the braid stability after implantation. Thus, we investigated frequency, predictive factors, and clinical impact of the phenomenon of FD braid deformation (FDBD).

Methods: Consecutive intracranial aneurysms (IAs) treated with various FDs, between January 2018 and July 2023, were reviewed to identify FDBD (defined as the deformation of a FD without any external force applied to it).

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Article Synopsis
  • The study examined the effectiveness of the Woven EndoBridge (WEB) device in sealing aneurysms by focusing on neck apposition and its relation to occlusion rates.
  • Researchers analyzed data from 159 aneurysms treated between March 2017 and May 2022, finding that good neck apposition led to a significantly higher likelihood of achieving complete and adequate occlusion.
  • The results suggested that minimizing WEB protrusion is important because it increased the risk of thromboembolic complications, even though it may have a limited effect on aneurysm occlusion rates.
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A decrease in Fkbp52 alters autophagosome maturation and A152T-tau clearance .

Front Cell Neurosci

July 2024

Institut Professeur Baulieu, INSERM U1195, Kremlin-Bicêtre, France.

The failure of the autophagy-lysosomal pathway to clear the pathogenic forms of Tau exacerbates the pathogenesis of tauopathies. We have previously shown that the immunophilin FKBP52 interacts both physically and functionally with Tau, and that a decrease in FKBP52 protein levels is associated with Tau deposition in affected human brains. We have also shown that FKBP52 is physiologically present within the lysosomal system in healthy human neurons and that a decrease in FKBP52 expression alters perinuclear lysosomal positioning and Tau clearance during Tau-induced proteotoxic stress .

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Effectiveness of patisiran after switching from tafamidis for the treatment of hereditary transthyretin-mediated amyloidosis with polyneuropathy.

Eur J Neurol

September 2024

Neurology Department, Assistance Publique-Hopitaux de Paris (AP-HP), CERAMIC, Centre Hospitalier Universitaire (CHU) Bicêtre, INSERM U1195, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

Article Synopsis
  • Hereditary transthyretin-mediated amyloidosis with polyneuropathy (ATTRv-PN) is a rare and progressing condition that causes various health issues, and this study looks at patients switching from tafamidis to patisiran treatment.
  • The research involved reviewing medical records of 24 patients who switched treatments, focusing on demographic data, reasons for the switch, and health outcomes over time.
  • Results showed that patients initially worsened on tafamidis but experienced slower disease progression and potential improvement after switching to patisiran, indicating the importance of timely treatment changes.
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Neurosteroids and translocator protein 18 kDa (TSPO) ligands as novel treatment options in depression.

Eur Arch Psychiatry Clin Neurosci

July 2024

Department of Psychiatry and Psychotherapy, University Regensburg, Universitätsstrasse 84, 93053, Regensburg, Germany.

Article Synopsis
  • The GABA system, specifically 3α-reduced steroids like allopregnanolone, has gained attention for treating anxiety and depression, particularly postpartum depression, with recent FDA approvals marking significant progress.
  • Brexanolone, an intravenous form of allopregnanolone, and zuranolone, an oral option, are both FDA-approved for postpartum depression, although zuranolone's approval for major depressive disorder is still pending due to insufficient efficacy data.
  • Side effects of these treatments include somnolence, dizziness, and headache, and current research is exploring alternatives like etifoxine, a TSPO ligand, which is already available in France for anxiety and may have potential antidepressant properties.
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French National Protocol for diagnosis and care of facioscapulohumeral muscular dystrophy (FSHD).

J Neurol

September 2024

Peripheral Nervous System and Muscle Department, Université Côte d'Azur, CHU Nice, Pasteur 2, Nice Hospital, France.

Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common genetically inherited myopathies in adults. It is characterized by incomplete penetrance and variable expressivity. Typically, FSHD patients display asymmetric weakness of facial, scapular, and humeral muscles that may progress to other muscle groups, particularly the abdominal and lower limb muscles.

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Background: Flow diverting stents (FDS) have transformed the treatment of intracranial aneurysms; however, their metallic structure associated with their intra-luminal positioning hamper angiographic and clinical outcomes. Therefore, there is a need to develop FDS with optimized surfaces that reduce thrombogenicity while promoting the healing process and endothelialization.

Methods: P8RI, a peptide mimicking the CD31 protein, was previously developed and grafted onto Silk Vista (SV) FDS.

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The New Zealand rabbit elastase-induced arterial aneurysm of the right common carotid artery remains a widely used model for assessing the effectiveness and safety of new neuroendovascular devices.1 This model offers a simple and reliable platform for pre-clinical investigations, crucial for comprehending the biological processes underlying aneurysm healing after endovascular treatment.2 Notably, the induced aneurysm exhibits morphological, hemodynamic, and histological characteristics similar to human intracranial aneurysms.

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Article Synopsis
  • Type 2 diabetes mellitus (T2DM) is linked to poor diet and lack of exercise, leading to insulin resistance, and personalized exercise programs may be more effective than standard ones for treatment.
  • A study involving various T2DM mouse models tested swimming-based exercise regimens tailored to their specific metabolic issues, revealing unique adaptations in muscle metabolism based on genetics and sex.
  • The findings suggested that these personalized exercises significantly improved metabolic health, reducing insulin resistance and enhancing glucose regulation in T2DM, highlighting the need for precision in exercise prescriptions for patients.
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Article Synopsis
  • The study analyzed 166 patients with acute neurological symptoms linked to anti-GQ1b antibodies, revealing frequent symptoms like areflexia, sensory issues, and muscle weakness.
  • The majority of patients were treated with intravenous immunoglobulins, leading to complete neurological recovery for 69% at the one-year mark, although 15% experienced relapses.
  • Key predictors for incomplete recovery included age over 70, initial ICU admission, and absence of anti-GQ1b antibodies; no predictors for relapse were identified.
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Nestorone® (segesterone acetate) is a progestin with a chemical structure closely related to progesterone with high affinity and selectivity for the progesterone receptor without significant interaction with other steroid receptors. It has been developed for female and male contraception and is FDA-approved in a first long-acting contraceptive vaginal system for female contraception. Its safety has been extensively demonstrated in both preclinical and clinical studies for contraceptive indications.

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Both Hedgehog and androgen signaling pathways are known to promote myelin regeneration in the central nervous system. Remarkably, the combined administration of agonists of each pathway revealed their functional cooperation towards higher regeneration in demyelination models in males. Since multiple sclerosis, the most common demyelinating disease, predominates in women, and androgen effects were reported to diverge according to sex, it seemed essential to assess the existence of such cooperation in females.

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Article Synopsis
  • * In a study, patients were divided based on the severity of their neuropathy at the beginning, and results indicated that those starting vutrisiran earlier, particularly with less severe neuropathy, had better outcomes across multiple health measures after 18 months.
  • * The analysis confirmed that vutrisiran helped improve neuropathy severity, quality of life, disability, gait speed, and nutritional status in patients, maintaining effectiveness regardless of their
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