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Congophilic angiopathies

Paweł P. Liberski

Affiliacja i adres do korespondencji
Aktualn Neurol 2005, 3 (5), p. 183-188
Congophilic angiopathies (CAA) are a group of neurodegenerations caused by amyloid deposition in the vessel walls. Amyloids are molecularly different and, as in all amyloidoses, CAA may be hereditary caused by mutations in a precursor for a given amyloid or sporadic of unknown cause. “Amyloid” is a generic terms to define diverse proteins of common physicochemical properties. In particular, they are congophilic (stained with Congo red), they fluorescent following staining with thioflavin S and they are composed of fibrils which are visible under transmission electron microscope. 
In this review we will cover: 
- dementia of BRI family: 
- familial British dementia, FBD (peptide ABri), 
- familial Danish dementia, FDD (peptide ADan); 
- hereditary cerebral haemorrhage with amyloidosis, Dutch type, HCHWA-D (peptide Ab); 
- hereditary cerebral haemorrhage with amyloidosis, Icelandic type, HCHWA-I (cystatin C); 
- CAA associated with Alzheimer disease (peptide Ab); 
- familial Finnish amyloidosis (gelsolin). 
Słowa kluczowe
congophilic angiopathies, familial British dementia, familial Danish dementia, familial Finnish amyloidosis, amyloid