Lymphangioleiomyomatosis: what do we know and what are we looking for?
Lymphangioleiomyomatosis (LAM) is a rare disease characterised by proliferation of abnormal smooth muscle-like cells (LAM cells) leading to progressive cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects predominantly females and can occur sporadically or in pat...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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European Respiratory Society
2011-03-01
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| Series: | European Respiratory Review |
| Subjects: | |
| Online Access: | http://err.ersjournals.com/content/20/119/034.full.pdf+html |
| _version_ | 1818236424793620480 |
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| author | O. Torre S. Harari J. Moss |
| author_facet | O. Torre S. Harari J. Moss |
| author_sort | O. Torre |
| collection | DOAJ |
| description | Lymphangioleiomyomatosis (LAM) is a rare disease characterised by proliferation of abnormal smooth muscle-like cells (LAM cells) leading to progressive cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects predominantly females and can occur sporadically or in patients with tuberous sclerosis complex. This review describes the recent progress in our understanding of the molecular pathogenesis of the disease and LAM cell biology. It also summarises current therapeutic approaches and the most promising areas of research for future therapeutic strategies. |
| first_indexed | 2024-12-12T12:09:39Z |
| format | Article |
| id | doaj.art-3a5d6428fd6c492e8688927fe694a8d2 |
| institution | Directory Open Access Journal |
| issn | 0905-9180 1600-0617 |
| language | English |
| last_indexed | 2024-12-12T12:09:39Z |
| publishDate | 2011-03-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | European Respiratory Review |
| spelling | doaj.art-3a5d6428fd6c492e8688927fe694a8d22022-12-22T00:24:56ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172011-03-01201193444Lymphangioleiomyomatosis: what do we know and what are we looking for?O. TorreS. HarariJ. MossLymphangioleiomyomatosis (LAM) is a rare disease characterised by proliferation of abnormal smooth muscle-like cells (LAM cells) leading to progressive cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects predominantly females and can occur sporadically or in patients with tuberous sclerosis complex. This review describes the recent progress in our understanding of the molecular pathogenesis of the disease and LAM cell biology. It also summarises current therapeutic approaches and the most promising areas of research for future therapeutic strategies.http://err.ersjournals.com/content/20/119/034.full.pdf+htmlLAM cellslymphangioleiomyomatosistuberous sclerosis complex |
| spellingShingle | O. Torre S. Harari J. Moss Lymphangioleiomyomatosis: what do we know and what are we looking for? European Respiratory Review LAM cells lymphangioleiomyomatosis tuberous sclerosis complex |
| title | Lymphangioleiomyomatosis: what do we know and what are we looking for? |
| title_full | Lymphangioleiomyomatosis: what do we know and what are we looking for? |
| title_fullStr | Lymphangioleiomyomatosis: what do we know and what are we looking for? |
| title_full_unstemmed | Lymphangioleiomyomatosis: what do we know and what are we looking for? |
| title_short | Lymphangioleiomyomatosis: what do we know and what are we looking for? |
| title_sort | lymphangioleiomyomatosis what do we know and what are we looking for |
| topic | LAM cells lymphangioleiomyomatosis tuberous sclerosis complex |
| url | http://err.ersjournals.com/content/20/119/034.full.pdf+html |
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