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Current Evidence Supporting the Role of Immune Response in ATTRv Amyloidosis
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Current Evidence Supporting the Role of Immune Response in ATTRv Amyloidosis
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Journal Article
Current Evidence Supporting the Role of Immune Response in ATTRv Amyloidosis
2023
Overview
Hereditary transthyretin (ATTRv) amyloidosis with polyneuropathy, also known as familial amyloid polyneuropathy (FAP), represents a progressive, heterogeneous, severe, and multisystemic disease caused by pathogenic variants in the TTR gene. This autosomal-dominant neurogenetic disorder has an adult onset with variable penetrance and an inconstant phenotype, even among subjects carrying the same mutation. Historically, ATTRv amyloidosis has been viewed as a non-inflammatory disease, mainly due to the absence of any mononuclear cell infiltration in ex vivo tissues; nevertheless, a role of inflammation in its pathogenesis has been recently highlighted. The immune response may be involved in the development and progression of the disease. Fibrillary TTR species bind to the receptor for advanced glycation end products (RAGE), probably activating the nuclear factor κB (NF-κB) pathway. Moreover, peripheral blood levels of several cytokines, including interferon (IFN)-gamma, IFN-alpha, IL-6, IL-7, and IL-33, are altered in the course of the disease. This review summarizes the current evidence supporting the role of the immune response in ATTRv amyloidosis, from the pathological mechanisms to the possible therapeutic implications.
Publisher
MDPI AG,MDPI
Subject
/ Advanced glycosylation end products
/ Amyloid
/ Amyloid Neuropathies, Familial
/ Cytology
/ familial amyloid polyneuropathy
/ hereditary transthyretin amyloidosi
/ hereditary transthyretin amyloidosis
/ hereditary transthyretin amyloidosis; polyneuropathy; familial amyloid polyneuropathy; inflammation
/ Humans
/ Immunity
/ Proteins
/ Receptor for Advanced Glycation End Products
/ Review
/ Toxicity
