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Lysosomal proteome analysis reveals that CLN3-defective cells have multiple enzyme deficiencies associated with changes in intracellular trafficking.
Schmidtke, Carolin; Tiede, Stephan; Thelen, Melanie; Käkelä, Reijo; Jabs, Sabrina; Makrypidi, Georgia; Sylvester, Marc; Schweizer, Michaela; Braren, Ingke; Brocke-Ahmadinejad, Nahal; Cotman, Susan L; Schulz, Angela; Gieselmann, Volkmar; Braulke, Thomas.
Afiliação
  • Schmidtke C; From the Department of Biochemistry, Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany 20246.
  • Tiede S; From the Department of Biochemistry, Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany 20246.
  • Thelen M; Institute of Biochemistry and Molecular Biology, University of Bonn, Bonn, Germany D-53115.
  • Käkelä R; Molecular and Integrative Biosciences Research Programme, University of Helsinki, Helsinki, Finland 00014.
  • Jabs S; Leibniz-Institut für Molekulare Pharmakologie (FMP) and Max-Delbrück-Centrum für Molekulare Medizin (MDC), Berlin, Germany 13125.
  • Makrypidi G; From the Department of Biochemistry, Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany 20246.
  • Sylvester M; Institute of Biochemistry and Molecular Biology, University of Bonn, Bonn, Germany D-53115.
  • Schweizer M; the Department of Electron Microscopy, Center for Molecular Neurobiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany 20251.
  • Braren I; Vector Core Unit, University Medical Center Hamburg-Eppendorf, Hamburg, Germany 20251.
  • Brocke-Ahmadinejad N; Institute of Biochemistry and Molecular Biology, University of Bonn, Bonn, Germany D-53115.
  • Cotman SL; Center for Genomic Medicine, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts 02114.
  • Schulz A; From the Department of Biochemistry, Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany 20246.
  • Gieselmann V; Institute of Biochemistry and Molecular Biology, University of Bonn, Bonn, Germany D-53115.
  • Braulke T; From the Department of Biochemistry, Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany 20246, braulke@uke.de.
J Biol Chem ; 294(24): 9592-9604, 2019 06 14.
Article em En | MEDLINE | ID: mdl-31040178
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Glicoproteínas de Membrana / Proteínas / Cerebelo / Proteoma / Transporte Proteico / Lipídeos / Lisossomos Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Animals Idioma: En Revista: J Biol Chem Ano de publicação: 2019 Tipo de documento: Article País de publicação: Estados Unidos
Texto completo
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Glicoproteínas de Membrana / Proteínas / Cerebelo / Proteoma / Transporte Proteico / Lipídeos / Lisossomos Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Animals Idioma: En Revista: J Biol Chem Ano de publicação: 2019 Tipo de documento: Article País de publicação: Estados Unidos