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


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 (PrP(C)). 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 PrP(C) using a well-established methodology based on the immunization of Prnp (0/0) mice. Additionally, we show their ability to block prion (PrP(Sc)) 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 PrP(C).

SUBMITTER: Didonna A 

PROVIDER: S-EPMC4369333 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature

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

Didonna Alessandro A   Venturini Anja Colja AC   Hartman Katrina K   Vranac Tanja T   Čurin Šerbec Vladka V   Legname Giuseppe G  

PeerJ 20150319


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 (PrP(C)). 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 di  ...[more]

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