Nerve pathology of microangiopathy and thromboinflammation in hereditary transthyretin amyloidosis.

ABSTRACT:

Objective

Despite amyloid deposition as a hallmark of hereditary transthyretin amyloidosis (ATTRv) with polyneuropathy, this pathology could not completely account for nerve degeneration. ATTRv patients frequently have vasomotor symptoms, but microangiopathy hypothesis in ATTRv was not systemically clarified.

Methods

This study examined the vascular pathology of sural nerves in ATTRv patients with transthyretin (TTR) mutation of p.Ala117Ser (TTR-A97S), focusing on morphometry and patterns of molecular expression in relation to nerve degeneration. We further applied human microvascular endothelial cell (HMEC-1) culture to examine the direct effect of TTR-A97S protein on endothelial cells.

Results

In ATTRv nerves, there was characteristic microangiopathy compared to con

SUBMITTER: Yeh SJ 

PROVIDER: S-EPMC10791016 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature