Prion diseases are a group of fatal neurodegenerative disorders that affect humans and animals. They are characterized by the accumulation in the central nervous system of a pathological form of the host-encoded prion protein (PrPC). The prion protein is a membrane glycoprotein that consists of two domains: a globular, structured C-terminus and an unstructured N-terminus. The N-terminal part of the protein is involved in different functions in both health and disease. In the present work we discuss the production and biochemical characterization of a panel of four monoclonal antibodies (mAbs) against the distal N-terminus of PrPC using a well-established methodology based on the immunization of Prnp0/0 mice. Additionally, we show their ability to block prion (PrPSc) replication at nanomolar concentrations in a cell culture model of prion infection. These mAbs represent a promising tool for prion diagnostics and for studying the physiological role of the N-terminal domain of PrPC.

Characterization of four new monoclonal antibodies against the distal N-terminal region of PrP(c) / Didonna, Alessandro; Venturini, A. C.; Hartman, K.; Vranac, T.; Ŝerbec, V. C.; Legname, Giuseppe. - In: PEERJ. - ISSN 2167-8359. - 2015:3(2015), pp. e811.1-e811.24. [10.7717/peerj.811]

Characterization of four new monoclonal antibodies against the distal N-terminal region of PrP(c)

Didonna, Alessandro;Legname, Giuseppe
2015-01-01

Abstract

Prion diseases are a group of fatal neurodegenerative disorders that affect humans and animals. They are characterized by the accumulation in the central nervous system of a pathological form of the host-encoded prion protein (PrPC). The prion protein is a membrane glycoprotein that consists of two domains: a globular, structured C-terminus and an unstructured N-terminus. The N-terminal part of the protein is involved in different functions in both health and disease. In the present work we discuss the production and biochemical characterization of a panel of four monoclonal antibodies (mAbs) against the distal N-terminus of PrPC using a well-established methodology based on the immunization of Prnp0/0 mice. Additionally, we show their ability to block prion (PrPSc) replication at nanomolar concentrations in a cell culture model of prion infection. These mAbs represent a promising tool for prion diagnostics and for studying the physiological role of the N-terminal domain of PrPC.
2015
2015
3
1
24
e811
10.7717/peerj.811
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4369333/
https://peerj.com/articles/811/
Didonna, Alessandro; Venturini, A. C.; Hartman, K.; Vranac, T.; Ŝerbec, V. C.; Legname, Giuseppe
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11767/15967
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