Abstract:
:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is characterized by the deposition of granular osmiophilic material in association with vascular smooth muscle cells in many different organs. However, the cause of the subsequent destruction of smooth muscle cells that are surrounded by granular osmiophilic material is unclear. In the present study, the ultrastructural changes that occur in endothelial cells in CADASIL have been evaluated by examining blood vessels in six skin biopsies and seven muscle biopsies belonging to three different CADASIL pedigrees. The appearances have been compared with five skin biopsies and five muscle biopsies from age-matched controls without vascular disease. The most striking features observed in vessels in the skin of CADASIL patients were attenuation of endothelial cells and increased density of endothelial cytoplasm, accompanied by the presence of compact bundles of microfilaments within the cytoplasm of endothelial cells. Endothelial cells in muscle biopsies from CADASIL patients, on the other hand, were swollen until destruction of tight junctions were observed. These findings suggest that impaired permeability of vascular endothelium may play a role in the destruction of vascular smooth muscle cells in CADASIL. Furthermore, the results of this study suggest that further fine structural investigation of blood vessel endothelium and underlying smooth muscle may lead to a better understanding of the pathophysiology of CADASIL.
journal_name
Neuropathol Appl Neurobioljournal_title
Neuropathology and applied neurobiologyauthors
Ruchoux MM,Maurage CAdoi
10.1046/j.1365-2990.1998.00087.xsubject
Has Abstractpub_date
1998-02-01 00:00:00pages
60-5issue
1eissn
0305-1846issn
1365-2990journal_volume
24pub_type
杂志文章abstract:AIMS:Frontotemporal lobar degeneration (FTLD) and motor neurone disease are linked by the possession of a hexanucleotide repeat expansion in C9ORF72, and both show neuronal cytoplasmic inclusions within cerebellar and hippocampal neurones which are TDP-43 negative but immunoreactive for p62 and dipeptide repeat protein...
journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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doi:10.1111/j.1365-2990.1992.tb00789.x
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journal_title:Neuropathology and applied neurobiology
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doi:10.1111/j.1365-2990.1983.tb00111.x
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journal_title:Neuropathology and applied neurobiology
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doi:10.1111/j.1365-2990.1994.tb00956.x
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/nan.12315
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journal_title:Neuropathology and applied neurobiology
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doi:
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journal_title:Neuropathology and applied neurobiology
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更新日期:2011-06-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.2003.00543.x
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1986.tb00047.x
更新日期:1986-03-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1990.tb00930.x
更新日期:1990-02-01 00:00:00
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更新日期:2010-04-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2018-02-01 00:00:00
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doi:10.1111/nan.12291
更新日期:2016-12-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1993.tb00458.x
更新日期:1993-10-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1046/j.1365-2990.1998.00114.x
更新日期:1998-06-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1992.tb00800.x
更新日期:1992-08-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
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更新日期:2021-02-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
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更新日期:1989-11-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
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更新日期:2001-06-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1994.tb00985.x
更新日期:1994-08-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1994.tb00995.x
更新日期:1994-10-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1986.tb00054.x
更新日期:1986-09-01 00:00:00
abstract::Brains from human fetuses of 13 to 27 weeks gestation have been examined immunohistologically for the presence of macrophages using the marker alpha-1-anti-chymotrypsin. A preliminary study demonstrated this to be a satisfactory marker of brain macrophages, although macrophages were also weakly positive for the more s...
journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1991.tb00738.x
更新日期:1991-10-01 00:00:00
abstract:AIMS:post-haemorrhagic ventricular dilatation (PHVD) is a significant problem in neonatal care, with sequelae extending beyond childhood. Its management is important in determining outcome. Although rodent hydrocephalus models have been developed, PHVD, as a specific entity with a distinct pathophysiology, has not been...
journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.2010.01118.x
更新日期:2011-02-01 00:00:00
abstract:AIMS:Brain mural cells (BMC), smooth muscle cells and pericytes, interact closely with endothelial cells and modulate numerous cerebrovascular functions. A loss of BMC function is suspected to play a role in the pathophysiology of Alzheimer's Disease (AD). METHODS:BMC markers, namely smooth muscle alpha actin (α-SMA) ...
journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/nan.12599
更新日期:2020-08-01 00:00:00