α-Synuclein Spread from Olfactory Bulb Causes Hyposmia, Anxiety, and Memory Loss in BAC-SNCA Mice.


Journal

Movement disorders : official journal of the Movement Disorder Society
ISSN: 1531-8257
Titre abrégé: Mov Disord
Pays: United States
ID NLM: 8610688

Informations de publication

Date de publication:
09 2021
Historique:
revised: 07 12 2020
received: 08 10 2020
accepted: 03 01 2021
pubmed: 7 2 2021
medline: 14 10 2021
entrez: 6 2 2021
Statut: ppublish

Résumé

Patients with Parkinson's disease (PD) show motor symptoms as well as various non-motor symptoms. Postmortem studies of PD have suggested that initial alpha-synuclein (α-Syn) pathology develops independently in the olfactory bulb and lower brainstem, spreading from there stereotypically. However, it remains unclear how these two pathological pathways contribute to the clinicopathological progression of PD. The objective of this study was to examine the clinicopathological contribution of α-Syn spread from the olfactory bulb. We conducted pathological and behavioral analyses of human α-Syn bacterial artificial chromosome transgenic mice injected with α-Syn preformed fibrils into the bilateral olfactory bulb up to 10 months postinjection. α-Syn preformed fibril injections induced more widespread α-Syn pathology in the transgenic mice than that in wild-type mice. Severe α-Syn pathology in the transgenic mice injected with α-Syn preformed fibrils was initially observed along the olfactory pathway and later in the brain regions that are included in the limbic system and have connections with it. The α-Syn pathology was accompanied by regional atrophy, neuron loss, reactive astrogliosis, and microglial activation, which were remarkable in the hippocampus. Behavioral analyses revealed hyposmia, followed by anxiety-like behavior and memory impairment, but not motor dysfunction, depression-like behavior, or circadian rhythm disturbance. Our data suggest that α-Syn spread from the olfactory bulb mainly affects the olfactory pathway and limbic system as well as its related regions, leading to the development of hyposmia, anxiety, and memory loss in PD. © 2021 International Parkinson and Movement Disorder Society.

Sections du résumé

BACKGROUND
Patients with Parkinson's disease (PD) show motor symptoms as well as various non-motor symptoms. Postmortem studies of PD have suggested that initial alpha-synuclein (α-Syn) pathology develops independently in the olfactory bulb and lower brainstem, spreading from there stereotypically. However, it remains unclear how these two pathological pathways contribute to the clinicopathological progression of PD.
OBJECTIVE
The objective of this study was to examine the clinicopathological contribution of α-Syn spread from the olfactory bulb.
METHODS
We conducted pathological and behavioral analyses of human α-Syn bacterial artificial chromosome transgenic mice injected with α-Syn preformed fibrils into the bilateral olfactory bulb up to 10 months postinjection.
RESULTS
α-Syn preformed fibril injections induced more widespread α-Syn pathology in the transgenic mice than that in wild-type mice. Severe α-Syn pathology in the transgenic mice injected with α-Syn preformed fibrils was initially observed along the olfactory pathway and later in the brain regions that are included in the limbic system and have connections with it. The α-Syn pathology was accompanied by regional atrophy, neuron loss, reactive astrogliosis, and microglial activation, which were remarkable in the hippocampus. Behavioral analyses revealed hyposmia, followed by anxiety-like behavior and memory impairment, but not motor dysfunction, depression-like behavior, or circadian rhythm disturbance.
CONCLUSION
Our data suggest that α-Syn spread from the olfactory bulb mainly affects the olfactory pathway and limbic system as well as its related regions, leading to the development of hyposmia, anxiety, and memory loss in PD. © 2021 International Parkinson and Movement Disorder Society.

Identifiants

pubmed: 33547846
doi: 10.1002/mds.28512
pmc: PMC8996681
mid: NIHMS1790322
doi:

Substances chimiques

SNCA protein, human 0
Snca protein, mouse 0
alpha-Synuclein 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

2036-2047

Subventions

Organisme : NIA NIH HHS
ID : U19 AG062418
Pays : United States

Informations de copyright

© 2021 International Parkinson and Movement Disorder Society.

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Auteurs

Norihito Uemura (N)

Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.

Jun Ueda (J)

Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Toru Yoshihara (T)

Institute of Laboratory Animals, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Masashi Ikuno (M)

Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Maiko T Uemura (MT)

Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.

Hodaka Yamakado (H)

Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Masahide Asano (M)

Institute of Laboratory Animals, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

John Q Trojanowski (JQ)

Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.

Ryosuke Takahashi (R)

Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

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