Myositis facilitates preclinical accumulation of pathological prion protein in muscle.

ABSTRACT:

Background

In human and animal prion diseases, pathological prion protein, PrPSc, as well as prion infectivity is mainly found in the central nervous system, but also in lymphoid organs and muscle. Pathophysiology of prion colonization of lymphoid organs has been studied intensively, yet how myositis influences prion accumulation in muscle is unknown.

Result

We have investigated the influence of myositis on PrPSc accumulation and prion infectivity in two distinct mouse models of experimental autoimmune myositis. Furthermore, we have addressed the relevance of PrPC expression in the lymphoreticular system in myositis by generating bone marrow chimeras.Here we show that myositis positively influences muscular PrPSc accumulation at preclinical time points and that PrPC-expressi

SUBMITTER: Neumann M 

PROVIDER: S-EPMC4046662 | biostudies-literature | 2013 Dec

REPOSITORIES: biostudies-literature