title: Comparison between the Icelandic and Dutch forms of hereditary cerebral amyloid angiopathy.
abstract: Hereditary cerebral amyloid angiopathy has been described in Icelandic and Dutch families. Although the clinical manifestations show similarities, biochemical characterization revealed that amyloid in the Icelandic patients consists of cystatin C and in the Dutch patients of beta-protein. Both diseases are caused by a single base mutation leading to the same amino acid (viz. glutamine). Furthermore, both cystatin C and the beta-protein precursor are protease inhibitors. Therefore, the mechanism of amyloidogenesis may be similar in both diseases. A comparison of clinical, pathological, genetic, and biochemical aspects of these two types of hereditary cerebral amyloid angiopathy is presented.
authors.affiliation: Department of Neurology, University Hospital, Leiden, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Haan
authors.forename: R A
authors.initials: RA
authors.lastname: Roos
mesh_terms: D016564:Amyloid beta-Protein Precursor / Q000235:genetics; D001921:Brain / Q000473:pathology; D016657:Cerebral Amyloid Angiopathy / Q000235:genetics* / Q000473:pathology; D002536:Cerebral Arteries / Q000473:pathology; D002543:Cerebral Hemorrhage / Q000235:genetics* / Q000473:pathology; D003431:Cross-Cultural Comparison; D006801:Humans; D007055:Iceland; D009410:Nerve Degeneration / Q000235:genetics; D009426:Netherlands
chemical_list: D016564:Amyloid beta-Protein Precursor