Prion Protein Paralog Doppel Protein Interacts with Alpha-2-Macroglobulin: A Plausible Mechanism for Doppel-Mediated Neurodegeneration

Stefano Benvegnù; Diego Franciotta; Josh Sussman; Angela Bachi; Elisabetta Zardini; Paola Torreri; Cedric Govaerts; Salvatore Pizzo; Giuseppe Legname

doi:10.1371/journal.pone.0005968

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Open Access

Peer-reviewed

Research Article

Prion Protein Paralog Doppel Protein Interacts with Alpha-2-Macroglobulin: A Plausible Mechanism for Doppel-Mediated Neurodegeneration

Stefano Benvegnù,

Affiliation Scuola Internazionale Superiore di Studi Avanzati - International School for Advanced Studies (SISSA-ISAS), Trieste, Italy
⨯
Diego Franciotta,

Affiliation IRCCS, Foundation, Neurological Institute C. Mondino, University of Pavia, Pavia, Italy
⨯
Josh Sussman,

Current address: ImClone Systems Incorporated, New York, New York, United States of America

Affiliation Institute for Neurodegenerative Diseases
⨯
Angela Bachi,

Affiliation DIBIT, San Raffaele Scientific Institute, Milan, Italy
⨯
Elisabetta Zardini,

Affiliation IRCCS, Foundation, Neurological Institute C. Mondino, University of Pavia, Pavia, Italy
⨯
Paola Torreri,

Affiliation National Centre for Rare Diseases, Istituto Superiore di Sanità, Roma, Italy
⨯
Cedric Govaerts,

Affiliations Institute for Neurodegenerative Diseases, Université Libre de Bruxelles, Bruxelles, Belgium
⨯
Salvatore Pizzo,

Affiliation Department of Pathology, Duke University Medical Center, Durham, North Carolina, United States of America
⨯
Giuseppe Legname

* E-mail: legname@sissa.it

Affiliations Scuola Internazionale Superiore di Studi Avanzati - International School for Advanced Studies (SISSA-ISAS), Trieste, Italy, Institute for Neurodegenerative Diseases, Department of Neurology, University of California San Francisco, San Francisco, California, United States of America
⨯

Prion Protein Paralog Doppel Protein Interacts with Alpha-2-Macroglobulin: A Plausible Mechanism for Doppel-Mediated Neurodegeneration

Stefano Benvegnù,
Diego Franciotta,
Josh Sussman,
Angela Bachi,
Elisabetta Zardini,
Paola Torreri,
Cedric Govaerts,
Salvatore Pizzo,
Giuseppe Legname

Published: June 18, 2009
https://doi.org/10.1371/journal.pone.0005968

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a2M interaction with PrP(89-230)?

Posted by loma on 18 Sep 2009 at 08:45 GMT

The paper presents very interesting findings and interpretation! What I missed is data on how N-terminally truncated PrP, e.g. recPrP(89-230), interacts with a2M. Rather akin to Dpl or to full-length PrP? If the former is the case, there may be an alternative interpretation of the scenario of Fig. 9E: neurodegenration occurs not because of inefficient sequestration of Dpl but due to (co-)sequestration of a2M by truncated PrP.

No competing interests declared.

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Prion Protein Paralog Doppel Protein Interacts with Alpha-2-Macroglobulin: A Plausible Mechanism for Doppel-Mediated Neurodegeneration

Prion Protein Paralog Doppel Protein Interacts with Alpha-2-Macroglobulin: A Plausible Mechanism for Doppel-Mediated Neurodegeneration

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a2M interaction with PrP(89-230)?

Posted by loma on 18 Sep 2009 at 08:45 GMT