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Stochastic Optical Reconstruction Microscopy Imaging of Multiple System Atrophy Inclusions Suggests Stepwise α-Synuclein Aggregation.
Vovard, Benoît; Bodin, Alexia; Gouju, Julien; de Guilhem de Lataillade, Adrien; Derkinderen, Pascal; Etcharry-Bouyx, Frédérique; Chauviré, Valérie; Guillet-Pichon, Virginie; Verny, Christophe; Letournel, Franck; Lenaers, Guy; Chevrollier, Arnaud; Codron, Philippe.
Afiliación
  • Vovard B; Univ Angers, Equipe MitoLab, Unité MitoVasc, Inserm U1083, CNRS 6015, SFR ICAT, Angers, France.
  • Bodin A; Laboratoire de neurobiologie et neuropathologie, Centre Hospitalier Universitaire d'Angers, Angers, France.
  • Gouju J; Univ Angers, Equipe MitoLab, Unité MitoVasc, Inserm U1083, CNRS 6015, SFR ICAT, Angers, France.
  • de Guilhem de Lataillade A; Laboratoire de neurobiologie et neuropathologie, Centre Hospitalier Universitaire d'Angers, Angers, France.
  • Derkinderen P; Laboratoire de neurobiologie et neuropathologie, Centre Hospitalier Universitaire d'Angers, Angers, France.
  • Etcharry-Bouyx F; MINT, UMR Inserm 1066, CNRS 6021, Angers, France.
  • Chauviré V; Université de Nantes, Inserm, TENS, The Enteric Nervous System in Gut and Brain Diseases, IMAD, Nantes, France.
  • Guillet-Pichon V; Service de Neurologie, CHU Nantes, Inserm U1235 Nantes, Nantes, France.
  • Verny C; Université de Nantes, Inserm, TENS, The Enteric Nervous System in Gut and Brain Diseases, IMAD, Nantes, France.
  • Letournel F; Service de Neurologie, CHU Nantes, Inserm U1235 Nantes, Nantes, France.
  • Lenaers G; Service de Neurologie, centre mémoire de ressource et de recherche, Centre Hospitalier Universitaire d'Angers, Angers, France.
  • Chevrollier A; Service de Neurologie, centre mémoire de ressource et de recherche, Centre Hospitalier Universitaire d'Angers, Angers, France.
  • Codron P; Service de Neurologie, centre mémoire de ressource et de recherche, Centre Hospitalier Universitaire d'Angers, Angers, France.
Mov Disord ; 39(4): 723-728, 2024 Apr.
Article en En | MEDLINE | ID: mdl-38357858
ABSTRACT

BACKGROUND:

The architecture and composition of glial (GCI) and neuronal (NCI) α-synuclein inclusions observed in multiple system atrophy (MSA) remain to be precisely defined to better understand the disease.

METHODS:

Here, we used stochastic optical reconstruction microscopy (STORM) to characterize the nanoscale organization of glial (GCI) and neuronal (NCI) α-synuclein inclusions in cryopreserved brain sections from MSA patients.

RESULTS:

STORM revealed a dense cross-linked internal structure of α-synuclein in all GCI and NCI. The internal architecture of hyperphosphorylated α-synuclein (p-αSyn) inclusions was similar in glial and neuronal cells, suggesting a common aggregation mechanism. A similar sequence of p-αSyn stepwise intracellular aggregation was defined in oligodendrocytes and neurons, starting from the perinuclear area and growing inside the cells. Consistent with this hypothesis, we found a higher mitochondrial density in GCI and NCI compared to oligodendrocytes and neurons from unaffected donors (P < 0.01), suggesting an active recruitment of the organelles during the aggregation process.

CONCLUSIONS:

These first STORM images of GCI and NCI suggest stepwise α-synuclein aggregation in MSA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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Texto completo: 1 Base de datos: MEDLINE Asunto principal: Cuerpos de Inclusión / Atrofia de Múltiples Sistemas / Alfa-Sinucleína / Neuronas Límite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Mov Disord Asunto de la revista: NEUROLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Francia

Texto completo: 1 Base de datos: MEDLINE Asunto principal: Cuerpos de Inclusión / Atrofia de Múltiples Sistemas / Alfa-Sinucleína / Neuronas Límite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Mov Disord Asunto de la revista: NEUROLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Francia