Ataxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III).

Kowalewski, Björn; Heimann, Peter; Ortkras, Theresa; Lüllmann-Rauch, Renate; Sawada, Tomo; Walkley, Steven U; Dierks, Thomas; Damme, Markus

Kowalewski, Björn; Heimann, Peter; Ortkras, Theresa; Lüllmann-Rauch, Renate; Sawada, Tomo; Walkley, Steven U; Dierks, Thomas; Damme, Markus.

Afiliação

Kowalewski B; Department of Chemistry, Biochemistry I, Bielefeld University, 33615 Bielefeld, Germany.
Heimann P; Molecular Neurobiology, Faculty of Biology, Bielefeld University, 33615 Bielefeld, Germany.
Ortkras T; Department of Chemistry, Biochemistry I, Bielefeld University, 33615 Bielefeld, Germany.
Lüllmann-Rauch R; Anatomical Institute, University of Kiel, D-24098 Kiel, Germany.
Sawada T; The Dominick P. Purpura Department of Neuroscience, Rose F. Kennedy Intellectual and Developmental Disabilities Research Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
Walkley SU; The Dominick P. Purpura Department of Neuroscience, Rose F. Kennedy Intellectual and Developmental Disabilities Research Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
Dierks T; Department of Chemistry, Biochemistry I, Bielefeld University, 33615 Bielefeld, Germany.
Damme M; Department of Chemistry, Biochemistry I, Bielefeld University, 33615 Bielefeld, Germany, mdamme@biochem.uni-kiel.de.

Hum Mol Genet ; 24(7): 1856-68, 2015 Apr 01.

Article em En | MEDLINE | ID: mdl-25452429

Assuntos

Arilsulfatases/deficiência; Ataxia/enzimologia; Mucopolissacaridose III/enzimologia; Animais; Arilsulfatases/genética; Ataxia/genética; Ataxia/metabolismo; Ataxia/patologia; Cerebelo/citologia; Cerebelo/metabolismo; Modelos Animais de Doenças; Feminino; Gliose/metabolismo; Glicolipídeos/metabolismo; Humanos; Masculino; Camundongos; Camundongos Knockout; Mucopolissacaridose III/genética; Mucopolissacaridose III/metabolismo; Mucopolissacaridose III/patologia; Células de Purkinje/metabolismo

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Arilsulfatases / Ataxia / Mucopolissacaridose III Tipo de estudo: Diagnostic_studies / Prognostic_studies Limite: Animals / Female / Humans / Male Idioma: En Ano de publicação: 2015 Tipo de documento: Article

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