Microvascular degeneration in hereditary cystatin C amyloid angiopathy of the brain
Average rating
Cast your vote
You can rate an item by clicking the amount of stars they wish to award to this item.
When enough users have cast their vote on this item, the average rating will also be shown.
Star rating
Your vote was cast
Thank you for your feedback
Thank you for your feedback
Issue Date
1997-01-01
Metadata
Show full item recordCitation
APMIS. 1997, 105(1):41-7Abstract
Hereditary cystatin C amyloid angiopathy (HCCAA), an autosomal dominant form of cerebral amyloid angiopathy (CAA) occurring primarily in Iceland, is characterized by a variant cystatin C amyloid deposition in the walls of cerebral parenchymal and leptomeningeal vessels. Cystatin C is also found to colocalize with amyloid beta/A4 protein in cerebral vessel walls of patients with Alzheimer's disease (AD), sporadic CAA, and hereditary cerebral hemorrhage with amyloidosis, Dutch type (HCHWA-D). The abundance of cystatin C deposition in cerebral blood vessel walls suggests that cellular elements of the vessel wall itself may play a role in its deposition. Microvascular changes in the brains of HCCAA patients were investigated by single- and double-label immunohistochemistry. We found that cystatin C amyloid immunoreactivity was present not only in cerebral cortical and leptomeningeal vessels, but also in white matter parenchymal vessels. Cystatin C deposition was more prominent in the media of parenchymal vessels and in the adventitia of leptomeningeal vessels. Smooth muscle (sm) cells were few or could not be identified within vessel walls showing extensive cystatin C deposition, suggesting progressive loss of these cells as cystatin C accumulates. However, in less severely affected vessels, cystatin C was present in cells that also had the phenotype of sm, suggesting that sm cells synthesize or process cystatin C. Cystatin C immunoreactivity was in addition, detected in some neuronal cell bodies throughout the cortex in patients with HCCAA and AD-related CAA. Our results indicate that cellular components of the vessel walls may play an important role in cystatin C deposition, as they do in beta/A4 deposition in AD-related CAA. Cystatin C deposition within the vascular media and adventitia, with associated vessel wall injury as manifested by sm cell loss, represents microvascular degeneration that leads to cerebral hemorrhage.Description
To access publisher full text version of this article. Please click on the hyperlink in Additional Links fieldae974a485f413a2113503eed53cd6c53
10.1111/j.1699-0463.1997.tb00538.x
Scopus Count
Collections
Related articles
- Immunoreactive A4 and gamma-trace peptide colocalization in amyloidotic arteriolar lesions in brains of patients with Alzheimer's disease.
- Authors: Vinters HV, Nishimura GS, Secor DL, Pardridge WM
- Issue date: 1990 Aug
- Secondary microvascular degeneration in amyloid angiopathy of patients with hereditary cerebral hemorrhage with amyloidosis, Dutch type (HCHWA-D).
- Authors: Vinters HV, Natté R, Maat-Schieman ML, van Duinen SG, Hegeman-Kleinn I, Welling-Graafland C, Haan J, Roos RA
- Issue date: 1998 Mar
- Co-localization of beta/A4 and cystatin C in cortical blood vessels in Dutch, but not in Icelandic hereditary cerebral hemorrhage with amyloidosis.
- Authors: Haan J, Maat-Schieman ML, van Duinen SG, Jensson O, Thorsteinsson L, Roos RA
- Issue date: 1994 May
- Cerebral amyloid angiopathy and dementia.
- Authors: Tian J, Shi J, Mann DM
- Issue date: 2004 Dec
- No mutations in cystatin C gene in cerebral amyloid angiopathy with cystatin C deposition.
- Authors: Nagai A, Kobayashi S, Shimode K, Imaoka K, Umegae N, Fujihara S, Nakamura M
- Issue date: 1998 Jan