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Familial Cerebral Amyloid Angiopathy

Subscribe to New Research on Familial Cerebral Amyloid Angiopathy

A familial disorder marked by AMYLOID deposits in the walls of small and medium sized blood vessels of CEREBRAL CORTEX and MENINGES.

Also Known As:

Cerebral Amyloid Angiopathy, Familial; Cerebral Amyloid Angiopathy, Genetic; Cerebral Amyloid Angiopathy, Hereditary; Dutch Type Hereditary Cerebral Amyloid Angiopathy; Hereditary Cerebral Amyloid Angiopathy; Hereditary Cerebral Amyloid Angiopathy, Dutch Type; Hereditary Cerebral Amyloid Angiopathy, Icelandic Type; Icelandic Type Amyloidosis; Icelandic Type Hereditary Cerebral Amyloid Angiopathy; Amyloidosis, Icelandic Type

Networked: 37 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Cerebral Hemorrhage
2.	Amyloid Plaque
3.	Amyloidosis
4.	Alzheimer Disease (Alzheimer's Disease)
5.	Dementia (Dementias)

Experts

1.	Thormodsson, Finnbogi R: 3 articles (05/2013 - 12/2007)
2.	Greenberg, Steven M: 2 articles (11/2008 - 07/2008)
3.	Rosand, Jonathan: 2 articles (11/2008 - 07/2008)
4.	Van Nostrand, William E: 2 articles (03/2007 - 07/2005)
5.	Iconomidou, Vassiliki A: 1 article (09/2015)
6.	Hamodrakas, Stavros J: 1 article (09/2015)
7.	Tsiolaki, Paraskevi L: 1 article (09/2015)
8.	Louros, Nikolaos N: 1 article (09/2015)
9.	Ingolfsdottir, Indiana Elin: 1 article (05/2013)
10.	Petersen, Petur Henry: 1 article (05/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Familial Cerebral Amyloid Angiopathy:

1.	Amyloid (Amyloid Fibrils)IBA 10/01/2001 - "However each type of hereditary cerebral amyloid angiopathy has individual clinical and histopathological features reflecting the severity of arterial involvement, the extent of amyloid deposition within or outside the central nervous system, and the association with other neurodegenarative changes." 10/21/2000 - "Mutation of the genes coding for amyloid substance constituents have been identified in diverse forms of familial cerebral amyloid angiopathy." 06/01/1996 - "Yamada's letter to the editor: an amyloid protein in familial cerebral amyloid angiopathy (British type)" 02/01/1996 - "An amyloid protein in familial cerebral amyloid angiopathy (British type)" 09/01/2015 - "The oligomerization of hCC is involved in the pathophysiology of a rare form of amyloidosis namely Icelandic hereditary cerebral amyloid angiopathy, in which an L68Q mutant is deposited as amyloid in brain arteries of young adults. "
2.	Cystatin CIBA 05/01/2013 - "A mutation in the human cystatin C gene leads to familial cerebral amyloid angiopathy. " 05/14/1993 - "Cystatin-C is an amyloidogenic protein causing hereditary cerebral haemorrhage with amyloidosis-Icelandic type (HCHWA-I). " 04/01/1988 - "[Possibly "familial" cerebral amyloid angiopathy in Japan: immunohistochemical identification of gamma-trace]." 04/01/2001 - "Distinct properties of wild-type and the amyloidogenic human cystatin C variant of hereditary cerebral hemorrhage with amyloidosis, Icelandic type." 09/01/1999 - "Cellular processing of the amyloidogenic cystatin C variant of hereditary cerebral hemorrhage with amyloidosis, Icelandic type."
3.	AntibodiesIBA 09/14/1992 - "We isolated and carried out a chemical analysis of the amyloid fibril protein from the leptomeningeal vessels of a case with non-hereditary cerebral amyloid angiopathy (CAA) showing dual immunohistochemical reactivity with antibodies to both beta-protein and cystatin C. " 01/01/1992 - "Immunohistochemical studies were performed on biopsy specimens using primary antibodies against portions of the Alzheimer A4 (beta-) peptide or gamma-trace peptide (the vascular amyloid protein in patients with hereditary cerebral hemorrhage with amyloidosis-Icelandic type). "
4.	Cysteine Proteases (Cysteine Protease)IBA 01/01/2006 - "A variant of the cysteine protease inhibitor, cystatin C, forms amyloid deposited in the cerebral vasculature of patients with hereditary cerebral hemorrhage with amyloidosis, Icelandic type (HCHWA-I), leading to cerebral hemorrhages early in life. " 04/29/1986 - "Hereditary cerebral amyloid angiopathy: the amyloid fibrils contain a protein which is a variant of cystatin C, an inhibitor of lysosomal cysteine proteases." 09/01/1999 - "The protein, a mutant of the cysteine protease inhibitor cystatin C, is the amyloid precursor protein in Hereditary Cerebral Hemorrhage with Amyloidosis--Icelandic type (HCHWA-I). "
5.	TubulinIBA 02/01/1999 - "The histological features of familial cerebral amyloid angiopathy (British type) with non-neuritic amyloid plaque formation (FAB) include deposition of amyloid, (supposedly associated with the C-terminal fragments of both alpha- and beta-tubulin), in small cerebral and spinal arteries, hippocampal amyloid plaques and neurofibrillary tangles (NFTs) as well as ischaemic white matter changes. " 02/06/1996 - "C-terminal fragments of alpha- and beta-tubulin form amyloid fibrils in vitro and associate with amyloid deposits of familial cerebral amyloid angiopathy, British type."
6.	Staphylococcal Protein A (A, Protein)IBA 04/29/1986 - "Hereditary cerebral amyloid angiopathy: the amyloid fibrils contain a protein which is a variant of cystatin C, an inhibitor of lysosomal cysteine proteases." 09/01/1999 - "The protein, a mutant of the cysteine protease inhibitor cystatin C, is the amyloid precursor protein in Hereditary Cerebral Hemorrhage with Amyloidosis--Icelandic type (HCHWA-I). "
7.	ApolipoproteinsIBA 05/01/1997 - "Dutch hereditary cerebral amyloid angiopathy: structural lesions and apolipoprotein E genotype." 02/01/1995 - "No protective effect of apolipoprotein E epsilon 2 allele in Dutch hereditary cerebral amyloid angiopathy."
8.	Amyloid angiopathyIBA 04/01/1996 - "Knowledge about molecular pathology of hereditary cystatin C amyloid angiopathy (HCCAA), also called hereditary cerebral hemorrhage with amyloidosis, Icelandic type, has increased greatly in the last decade. " 05/01/2013 - "This disease is known as "hereditary cerebral hemorrhage with amyloidosis-Icelandic type" or "hereditary cystatin C amyloid angiopathy." The mutant cystatin C protein forms aggregates and amyloid, within the central nervous system almost exclusively in connection with the vascular system. "
9.	2- (4'- (methylamino)phenyl)- 6- hydroxybenzothiazole (Pittsburgh compound B)IBA 11/01/2008 - "We performed positron emission tomographic imaging with Pittsburgh Compound B on 42-year-old man with early manifestations of Iowa-type hereditary cerebral amyloid angiopathy, a form of the disorder with little or no plaque deposits of fibrillar beta-amyloid. " 04/01/2008 - "To investigate amyloid accumulation by carbon 11-labeled Pittsburgh Compound B (11C-PiB) in hereditary cerebral amyloid angiopathy and APP locus duplication. "
10.	glucuronyl glucosamine glycan sulfate (Vessel)IBA 12/01/2007 - "An aggressive and extremely rare form of CAA, known as Hereditary Cerebral Hemorrhage With Amyloidosis-Icelandic Type (HCHWA-I), exhibits this withdrawal of VSMC as amyloid accumulates in the vessel wall. " 04/01/1988 - "Immunoperoxidase on formalin-fixed, paraffin-embedded tissue sections from Alzheimer's Disease, Down's Syndrome, asymptomatic age-related cerebral amyloidosis, sporadic cerebral amyloid angiopathy, hereditary cerebral hemorrhage with amyloidosis-Icelandic type, and hereditary cerebral hemorrhage with amyloidosis-Dutch type revealed the presence of AP in the affected vessel walls in all cases, and in parenchymal deposits resembling neuritic plaques of Alzheimer's disease, sporadic cerebral amyloid angiopathy, and hereditary cerebral hemorrhage with amyloidosis-Dutch type. "