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amyloid polyneuropathy-nephropathy Iowa type; amyloidosis van Allen type; familial amyloid polyneuropathy type III (AMYLIOWA)
Pathology:
- hereditary generalized amyloidosis due to deposition of amyloid mainly constituted by apolipoprotein A1
Genetics:
- result of a mutation in APOA1
Clinical manifestations:
- neuropathy in the early stages of the disease
- nephropathy late in the course
- severe peptic ulcer disease can occur
- hearing loss is frequent
- cataracts may be present
- vitreous opacities are not observed
Complications:
- death is due in most cases to renal amyloidosis
General
amyloidosis
References
UniProt :accession P02647