Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.
Identifieur interne : 001B61 ( Ncbi/Merge ); précédent : 001B60; suivant : 001B62Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.
Auteurs : Anna Migdalska-Richards [Royaume-Uni] ; Liam Daly [Royaume-Uni] ; Erwan Bezard [France] ; Anthony H V. Schapira [Royaume-Uni]Source :
- Annals of neurology [ 1531-8249 ] ; 2016.
Abstract
Gaucher disease is caused by mutations in the glucocerebrosidase 1 gene that result in deficiency of the lysosomal enzyme glucocerebrosidase. Both homozygous and heterozygous glucocerebrosidase 1 mutations confer an increased risk for developing Parkinson disease. Current estimates indicate that 10 to 25% of Parkinson patients carry glucocerebrosidase 1 mutations. Ambroxol is a small molecule chaperone that has been shown to increase glucocerebrosidase activity in vitro. This study investigated the effect of ambroxol treatment on glucocerebrosidase activity and on α-synuclein and phosphorylated α-synuclein protein levels in mice.
DOI: 10.1002/ana.24790
PubMed: 27859541
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<front><div type="abstract" xml:lang="en">Gaucher disease is caused by mutations in the glucocerebrosidase 1 gene that result in deficiency of the lysosomal enzyme glucocerebrosidase. Both homozygous and heterozygous glucocerebrosidase 1 mutations confer an increased risk for developing Parkinson disease. Current estimates indicate that 10 to 25% of Parkinson patients carry glucocerebrosidase 1 mutations. Ambroxol is a small molecule chaperone that has been shown to increase glucocerebrosidase activity in vitro. This study investigated the effect of ambroxol treatment on glucocerebrosidase activity and on α-synuclein and phosphorylated α-synuclein protein levels in mice.</div>
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<Title>Annals of neurology</Title>
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<ArticleTitle>Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.</ArticleTitle>
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<Abstract><AbstractText Label="OBJECTIVE" NlmCategory="OBJECTIVE">Gaucher disease is caused by mutations in the glucocerebrosidase 1 gene that result in deficiency of the lysosomal enzyme glucocerebrosidase. Both homozygous and heterozygous glucocerebrosidase 1 mutations confer an increased risk for developing Parkinson disease. Current estimates indicate that 10 to 25% of Parkinson patients carry glucocerebrosidase 1 mutations. Ambroxol is a small molecule chaperone that has been shown to increase glucocerebrosidase activity in vitro. This study investigated the effect of ambroxol treatment on glucocerebrosidase activity and on α-synuclein and phosphorylated α-synuclein protein levels in mice.</AbstractText>
<AbstractText Label="METHODS" NlmCategory="METHODS">Mice were treated with ambroxol for 12 days. After the treatment, glucocerebrosidase activity was measured in the mouse brain lysates. The brain lysates were also analyzed for α-synuclein and phosphorylated α-synuclein protein levels.</AbstractText>
<AbstractText Label="RESULTS" NlmCategory="RESULTS">Ambroxol treatment resulted in increased brain glucocerebrosidase activity in (1) wild-type mice, (2) transgenic mice expressing the heterozygous L444P mutation in the murine glucocerebrosidase 1 gene, and (3) transgenic mice overexpressing human α-synuclein. Furthermore, in the mice overexpressing human α-synuclein, ambroxol treatment decreased both α-synuclein and phosphorylated α-synuclein protein levels.</AbstractText>
<AbstractText Label="INTERPRETATION" NlmCategory="CONCLUSIONS">Our work supports the proposition that ambroxol should be further investigated as a potential novel disease-modifying therapy for treatment of Parkinson disease and neuronopathic Gaucher disease to increase glucocerebrosidase activity and decrease α-synuclein and phosphorylated α-synuclein protein levels. Ann Neurol 2016;80:766-775.</AbstractText>
<CopyrightInformation>© 2016 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.</CopyrightInformation>
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