Abstract
Hereditary transthyrethin amyloidosis (ATTRv, v for variant) is a systemic disease with predominant involvement of the peripheral nervous system and heart. Other organs, such as eyes, gastrointestinal tract and kidney are frequently involved [1]. ATTRv has an autosomal-dominant mode of transmission and is characterized by deposition in the affected organs of insoluble misfolded protein fibrils derived from unstable transthyretin (TTR) tetramers, generated by missense mutations in the protein.
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