Differential expression of Cathepsin E in transthyretin amyloidosis: from neuropathology to the immune system.

ABSTRACT:

Background

Increasing evidence supports a key role for inflammation in the neurodegenerative process of familial amyloidotic polyneuropathy (FAP). While there seems to be an overactivation of the neuronal interleukin-1 signaling pathway, the immune response is apparently compromised in FAP. Accordingly, little immune cell infiltration is observed around pre-fibrillar or fibrillar amyloid deposits, with the underlying mechanism for this phenomenon remaining poorly understood. Cathepsin E (CtsE) is an important intermediate for antigen presentation and chemotaxis, but its role in the pathogenesis of FAP disease remains unknown.

Methods

In this study, we used both mouse primary macrophages and in vivo studies based on transgenic models of FAP and human samples to characterize C

SUBMITTER: Goncalves NP 

PROVIDER: S-EPMC5460450 | biostudies-literature | 2017 Jun

REPOSITORIES: biostudies-literature