Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis.
To access publisher's full text version of this article click on the hyperlink at the bottom of the page 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...
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Online Access: | http://hdl.handle.net/2336/322354 https://doi.org/10.1016/j.brainres.2013.08.029 |
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ftlandspitaliuni:oai:www.hirsla.lsh.is:2336/322354 2023-05-15T16:51:28+02:00 Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. Snorradottir, Asbjorg Osk Isaksson, Helgi J Kaeser, Stephan A Skodras, Angelos A Olafsson, Elias Palsdottir, Astridur Bragason, Birkir Thor Univ Iceland, Inst Expt Pathol Keldur, IS-112 Reykjavik, Iceland, Landspitali Univ Hosp, Dept Pathol, Reykjavik, Iceland, Univ Tubingen, Hertie Inst Clin Brain Res, Dept Cellular Neurol, Tubingen, Germany, German Ctr Neurodegenerat Dis, DZNE, Tubingen, Germany, Univ Iceland, Fac Med, IS-112 Reykjavik, Iceland, Landspitali Univ Hosp, Dept Neurol, Reykjavik, Iceland 2014 http://hdl.handle.net/2336/322354 https://doi.org/10.1016/j.brainres.2013.08.029 en eng Elsevier Science BV http://dx.doi.org/10.1016/j.brainres.2013.08.029 Brain Res. 2013, 1535:106-14 1872-6240 23973860 doi:10.1016/j.brainres.2013.08.029 http://hdl.handle.net/2336/322354 Brain research Archived with thanks to Brain research National Consortium - Landsaðgangur Heilablóðfall Stökkbreytingar Frumur Heilinn Fullorðnir Adult Aged 80 and over Aggrecans Amyloidosis Brain Cerebral Hemorrhage Collagen Type IV Cystatin C Female Humans Male Middle Aged Mutation Article 2014 ftlandspitaliuni https://doi.org/10.1016/j.brainres.2013.08.029 2022-05-29T08:21:56Z To access publisher's full text version of this article click on the hyperlink at the bottom of the page 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 the disease. The aim of this study was to increase understanding of the cerebral vascular pathology of HCCAA patients with an emphasis on structural changes within the arterial wall of affected leptomeningeal arteries. Examination of post-mortem samples revealed extensive changes in the walls of affected arteries characterised by deposition of extracellular matrix constituents, notably collagen IV and the proteoglycan aggrecan. Other structural abnormalities were thickening of the laminin distribution, intimal thickening concomitant with a frayed elastic layer, and variable reduction in the integrity of endothelia. Our results show that excess deposition of extracellular matrix proteins in cerebral arteries of HCCAA is a prominent feature of the disease and may play an important role in its pathogenesis. Icelandic Centre for Research (RANNIS) University of Iceland Research Fund Icelandic Centre for Research - Student's Innovation Fund Heilavernd fund Memorial fund of Hafdis Kjartansdottir Memorial fund of Helga Jonsdottir and Sigurlidi Kristjansson Article in Journal/Newspaper Iceland Hirsla - Landspítali University Hospital research archive Brain Research 1535 106 114 |
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Open Polar |
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Hirsla - Landspítali University Hospital research archive |
op_collection_id |
ftlandspitaliuni |
language |
English |
topic |
Heilablóðfall Stökkbreytingar Frumur Heilinn Fullorðnir Adult Aged 80 and over Aggrecans Amyloidosis Brain Cerebral Hemorrhage Collagen Type IV Cystatin C Female Humans Male Middle Aged Mutation |
spellingShingle |
Heilablóðfall Stökkbreytingar Frumur Heilinn Fullorðnir Adult Aged 80 and over Aggrecans Amyloidosis Brain Cerebral Hemorrhage Collagen Type IV Cystatin C Female Humans Male Middle Aged Mutation Snorradottir, Asbjorg Osk Isaksson, Helgi J Kaeser, Stephan A Skodras, Angelos A Olafsson, Elias Palsdottir, Astridur Bragason, Birkir Thor Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. |
topic_facet |
Heilablóðfall Stökkbreytingar Frumur Heilinn Fullorðnir Adult Aged 80 and over Aggrecans Amyloidosis Brain Cerebral Hemorrhage Collagen Type IV Cystatin C Female Humans Male Middle Aged Mutation |
description |
To access publisher's full text version of this article click on the hyperlink at the bottom of the page 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 the disease. The aim of this study was to increase understanding of the cerebral vascular pathology of HCCAA patients with an emphasis on structural changes within the arterial wall of affected leptomeningeal arteries. Examination of post-mortem samples revealed extensive changes in the walls of affected arteries characterised by deposition of extracellular matrix constituents, notably collagen IV and the proteoglycan aggrecan. Other structural abnormalities were thickening of the laminin distribution, intimal thickening concomitant with a frayed elastic layer, and variable reduction in the integrity of endothelia. Our results show that excess deposition of extracellular matrix proteins in cerebral arteries of HCCAA is a prominent feature of the disease and may play an important role in its pathogenesis. Icelandic Centre for Research (RANNIS) University of Iceland Research Fund Icelandic Centre for Research - Student's Innovation Fund Heilavernd fund Memorial fund of Hafdis Kjartansdottir Memorial fund of Helga Jonsdottir and Sigurlidi Kristjansson |
author2 |
Univ Iceland, Inst Expt Pathol Keldur, IS-112 Reykjavik, Iceland, Landspitali Univ Hosp, Dept Pathol, Reykjavik, Iceland, Univ Tubingen, Hertie Inst Clin Brain Res, Dept Cellular Neurol, Tubingen, Germany, German Ctr Neurodegenerat Dis, DZNE, Tubingen, Germany, Univ Iceland, Fac Med, IS-112 Reykjavik, Iceland, Landspitali Univ Hosp, Dept Neurol, Reykjavik, Iceland |
format |
Article in Journal/Newspaper |
author |
Snorradottir, Asbjorg Osk Isaksson, Helgi J Kaeser, Stephan A Skodras, Angelos A Olafsson, Elias Palsdottir, Astridur Bragason, Birkir Thor |
author_facet |
Snorradottir, Asbjorg Osk Isaksson, Helgi J Kaeser, Stephan A Skodras, Angelos A Olafsson, Elias Palsdottir, Astridur Bragason, Birkir Thor |
author_sort |
Snorradottir, Asbjorg Osk |
title |
Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. |
title_short |
Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. |
title_full |
Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. |
title_fullStr |
Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. |
title_full_unstemmed |
Deposition of collagen IV and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. |
title_sort |
deposition of collagen iv and aggrecan in leptomeningeal arteries of hereditary brain haemorrhage with amyloidosis. |
publisher |
Elsevier Science BV |
publishDate |
2014 |
url |
http://hdl.handle.net/2336/322354 https://doi.org/10.1016/j.brainres.2013.08.029 |
genre |
Iceland |
genre_facet |
Iceland |
op_relation |
http://dx.doi.org/10.1016/j.brainres.2013.08.029 Brain Res. 2013, 1535:106-14 1872-6240 23973860 doi:10.1016/j.brainres.2013.08.029 http://hdl.handle.net/2336/322354 Brain research |
op_rights |
Archived with thanks to Brain research National Consortium - Landsaðgangur |
op_doi |
https://doi.org/10.1016/j.brainres.2013.08.029 |
container_title |
Brain Research |
container_volume |
1535 |
container_start_page |
106 |
op_container_end_page |
114 |
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1766041581930938368 |