Article
Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis.
Brain research - 16 Oct 2013
Snorradottir Asbjorg Osk, Isaksson Helgi J, Kaeser Stephan A, Skodras Angelos A, Olafsson Elias, Palsdottir Astridur, Bragason Birkir Thor
Abstract excerpt
Hereditary Cystatin C Amyloid Angiopathy (HCCAA) is a rare genetic disease in Icelandic families caused by a mutation in the cystatin C gene, CST3. HCCAA is classified as a cerebral amyloid angiopathy and mutant cystatin C forms amyloid deposits in cerebral arteries resulting in fatal haemorrhagic strokes in young adults. The aetiology of HCCAA pathology is not clear and there is, at present, no animal model of...
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