Kongofilne angiopatie

• Authors: Paweł P. Liberski

Title variants

EN

Congophilic angiopathies

• Languages of publication: EN PL

Abstracts

EN

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 Aβ); • hereditary cerebral haemorrhage with amyloidosis, Icelandic type, HCHWA-I (cystatin C); • CAA associated with Alzheimer disease (peptide Aβ); • familial Finnish amyloidosis (gelsolin).

PL

Kongofilne angiopatie (ang. congophilic angiopathy, CAA) stanowią grupę chorób neurozwyrodnieniowych charakteryzujących się odkładaniem w ścianach naczyń ośrodkowego układu nerwowego (OUN) amyloidu o różnym składzie molekularnym. Jak wszystkie amyloidozy, CAA występują zarówno jako formy dziedziczne, uwarunkowane mutacjami w genach kodujących prekursor amyloidu, jak i postaci sporadyczne. W niniejszym przeglądzie zostaną omówione: - otępienia typu BRI: - rodzinne otępienie brytyjskie (ang. familial British dementia, FBD), - rodzinne otępienie duńskie (ang. familial Danish dementia, FDD); - dziedziczny krwotok mózgowy z amyloidozą typu holenderskiego (ang. hereditary cerebral haemorrhage with amyloidosis, Dutch type, HCHWA-D); - dziedziczny krwotok mózgowy z amyloidozą typu islandzkiego (ang. hereditary cerebral haemorrhage with amyloidosis, Icelandic type, HCHWA-I); - CAA towarzysząca chorobie Alzheimera; - CAA spowodowana odkładaniem gelsoliny – amyloidoza rodzinna typu fińskiego. We wszystkich tych chorobach występuje odkładanie się wokół naczyń amyloidów. Amyloidy są heterogenną grupą białek posiadających wspólne właściwości fizykochemiczne: kongofilię (barwienie czerwienią Kongo), fluorescencje po zabarwieniu tioflawiną S oraz tworzenie włókien obserwowanych w mikroskopie elektronowym. Amyloidy powstają z białek prekursorowych w wyniku ograniczonej proteolizy i zmiany konformacji z a-helikalnej na b-kartkę.

Keywords

EN

amyloid; amyloidoza rodzinna typu fińskiego; congophilic angiopathies; familial British dementia; familial Danish dementia; familial Finnish amyloidosis; kongofilne angiopatie; rodzinne otępienie brytyjskie; rodzinne otępienie duńskie

Discipline

• MEDICINE: MEDICINE

• Publisher: Medical Communications
• Journal: Aktualności Neurologiczne
• Year: 2005
• Volume: 5
• Issue: 3
• Pages: 183-188

Contributors

author

Paweł P. Liberski

• Zakład Patologii Molekularnej i Neuropatologii Katedry Onkologii Uniwersytetu Medycznego w Łodzi, ul. Czechosłowacka 8/10, 92-216 Łódź

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• Document Type: article