In Human and Mouse Spino-Cerebellar Tissue, Ataxin-2 Expansion Affects Ceramide-Sphingomyelin Metabolism.

Sen, Nesli-Ece; Arsovic, Aleksandar; Meierhofer, David; Brodesser, Susanne; Oberschmidt, Carola; Canet-Pons, Júlia; Kaya, Zeynep-Ece; Halbach, Melanie-Vanessa; Gispert, Suzana; Sandhoff, Konrad; Auburger, Georg

Sen, Nesli-Ece; Arsovic, Aleksandar; Meierhofer, David; Brodesser, Susanne; Oberschmidt, Carola; Canet-Pons, Júlia; Kaya, Zeynep-Ece; Halbach, Melanie-Vanessa; Gispert, Suzana; Sandhoff, Konrad; Auburger, Georg.

Afiliação

Sen NE; Experimental Neurology, Building 89, Goethe University Medical Faculty, Theodor Stern Kai 7, 60590 Frankfurt am Main, Germany.
Arsovic A; Faculty of Biosciences, Goethe-University, 60438, Frankfurt am Main, Germany.
Meierhofer D; Experimental Neurology, Building 89, Goethe University Medical Faculty, Theodor Stern Kai 7, 60590 Frankfurt am Main, Germany.
Brodesser S; Max Planck Institute for Molecular Genetics, Ihnestrasse 63-73, 14195 Berlin, Germany.
Oberschmidt C; Membrane Biology and Lipid Biochemistry Unit, Life and Medical Sciences Institute, University of Bonn, Bonn, Germany.
Canet-Pons J; Experimental Neurology, Building 89, Goethe University Medical Faculty, Theodor Stern Kai 7, 60590 Frankfurt am Main, Germany.
Kaya ZE; Experimental Neurology, Building 89, Goethe University Medical Faculty, Theodor Stern Kai 7, 60590 Frankfurt am Main, Germany.
Halbach MV; Experimental Neurology, Building 89, Goethe University Medical Faculty, Theodor Stern Kai 7, 60590 Frankfurt am Main, Germany.
Gispert S; Cerrahpasa School of Medicine, Istanbul University, 34098 Istanbul, Turkey.
Sandhoff K; Experimental Neurology, Building 89, Goethe University Medical Faculty, Theodor Stern Kai 7, 60590 Frankfurt am Main, Germany.
Auburger G; Experimental Neurology, Building 89, Goethe University Medical Faculty, Theodor Stern Kai 7, 60590 Frankfurt am Main, Germany.

Int J Mol Sci ; 20(23)2019 Nov 21.

Article em En | MEDLINE | ID: mdl-31766565

Assuntos

Ataxina-2/genética; Ceramidas/metabolismo; Esfingomielinas/metabolismo; Ataxias Espinocerebelares/genética; Expansão das Repetições de Trinucleotídeos/genética; Animais; Ataxina-2/metabolismo; Modelos Animais de Doenças; Proteínas do Olho/genética; Proteínas do Olho/metabolismo; Humanos; Metabolismo dos Lipídeos/genética; Proteínas de Membrana/genética; Proteínas de Membrana/metabolismo; Camundongos Knockout; Esfingomielina Fosfodiesterase/genética; Esfingomielina Fosfodiesterase/metabolismo; Esfingosina N-Aciltransferase/genética; Esfingosina N-Aciltransferase/metabolismo; Ataxias Espinocerebelares/metabolismo; Ataxias Espinocerebelares/patologia

Palavras-chave

SCA34; SCA38; Smpd1); acid sphingomyelinase (ASMase; amyotrophic lateral sclerosis (ALS); ceramide synthase (CERS2/CERS1); fatty acid elongase (Elovl1/4/5); leukodystrophy; neutral ceramidase (Asah2); neutral sphingomyelinase (Smpd3); olivo-ponto-cerebellar atrophy (OPCA); serine palmitoyltransferase 2 (Sptlc2)

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Esfingomielinas / Ceramidas / Expansão das Repetições de Trinucleotídeos / Ataxias Espinocerebelares / Ataxina-2 Limite: Animals / Humans Idioma: En Revista: Int J Mol Sci Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Alemanha

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