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Press Release: How toxic protein aggregates develop

Protein aggregates are deemed to be one reason for the death of nerve cells in disorders such as Alzheimer's or Huntington's disease. As researchers at the Max Planck Institute of Biochemistry report in the current issue of "Nature", they have now decoded a new cellular mechanism for the development of aggregates. Missing stop signals in the production of proteins lead erroneously to long lysine chains at the end of the protein. This in turn blocks the ribosomes, the cell's protein factory. Healthy cells detect blocked ribosomes and rapidly destroy useless proteins. If the necessary quality control machinery does not function properly, defective proteins accumulate and form toxic aggregates.

29.02.2016

In order to be able to treat neurodegenerative disorders in future, SyNergy member Ulrich Hartl, Head of the Department of Cellular Biochemistry at the Max Planck Institute of Biochemistry, and his team have for many years been studying the cellular causes for the death of nerve cells. A determining cause is believed to be protein deposits – aggregates of misfolded proteins. "We were able to show that the formation of aggregates is promoted by defects in the protein blueprint and these are not detected by the internal quality control machinery", explains Young-Jun Choe, first author of the study together with Sae-Hun Park.

Press release of the Max Planck Institute of Biochemistry

Um neurodegenerative Krankheiten in Zukunft behandeln zu können, untersucht SyNergy-Mitglied Ulrich Hartl, Leiter der Abteilung „Zelluläre Biochemie” am Max-Planck-Institut für Biochemie, zusammen mit seinem Team seit vielen Jahren die zellulären Ursachen für das Sterben von Nervenzellen. Als eine entscheidende Ursache gelten Proteinverklumpungen – Aggregate aus fehlgefalteten Proteinen. „Wir konnten zeigen, dass die Bildung von Aggregaten begünstigt wird, wenn es Fehler in der Proteinbauanleitung gibt und diese durch die interne Qualitätskontrolle nicht erkannt werden”, erklärt Young-Jun Choe, Erstautor der Studie neben Sae-Hun Park.

Pressemeldung des Max-Planck-Instituts für Biochemie