Mitochondrial dysfunction and calcium dysregulation in COQ8A-ataxia Purkinje neurons are rescued by CoQ10 treatment.

Manolaras, Ioannis; Del Bondio, Andrea; Griso, Olivier; Reutenauer, Laurence; Eisenmann, Aurélie; Habermann, Bianca H; Puccio, Hélène

Manolaras, Ioannis; Del Bondio, Andrea; Griso, Olivier; Reutenauer, Laurence; Eisenmann, Aurélie; Habermann, Bianca H; Puccio, Hélène.

Afiliação

Manolaras I; Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), Department of translational medecine and neurogenetics, 67404 Illkirch, France.
Del Bondio A; Inserm, U1258, 67404 Illkirch, France.
Griso O; CNRS, UMR7104, 67404 Illkirch, France.
Reutenauer L; Université de Strasbourg, 67000 Strasbourg, France.
Eisenmann A; Institut Neuromyogène, Pathophysiology and genetics of the neuron and muscle, Inserm U1315, 69008 Lyon, France.
Habermann BH; CNRS, Université Claude Bernard Lyon I, UMR 5261, 69008 Lyon, France.
Puccio H; Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), Department of translational medecine and neurogenetics, 67404 Illkirch, France.

Brain ; 146(9): 3836-3850, 2023 09 01.

Article em En | MEDLINE | ID: mdl-36960552

Assuntos

Ataxia Cerebelar; Camundongos; Animais; Ataxia Cerebelar/tratamento farmacológico; Ataxia Cerebelar/genética; Ataxia Cerebelar/metabolismo; Células de Purkinje/patologia; Cálcio/metabolismo; Ataxia/tratamento farmacológico; Ataxia/genética; Ataxia/metabolismo; Mitocôndrias/metabolismo

Palavras-chave

ataxia; calcium; coenzyme Q10; mitochondria; purkinje neurons

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Ataxia Cerebelar Limite: Animals Idioma: En Revista: Brain Ano de publicação: 2023 Tipo de documento: Article País de afiliação: França

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