Animal models for the mechanistic study of systemic lymphangiomatosis. 2011

Stanley G Rockson
Stanford University School of Medicine, Stanford, CA 94305, USA. srockson@cvmed.stanford.edu

The systematic study of focused animal models has produced an explosion of information regarding the mechanisms governing lymphatic development and the diseases associated with lymphatic dysfunction. Nevertheless, the pathogenesis of systemic lymphangiomatosis has, thus far, eluded mechanistic comprehension. In this review, recent molecular advances in lymphatic vascular development are considered within the context of the animal models that have produced evolving insights. The emerging role of the zebrafish within lymphatic investigation is discussed. Specific models of the human disease pathology are considered in detail. While much has been learned about the molecular framework that surrounds normal lymphatic vascular development, the defect responsible for systemic lymphangiomatosis remains elusive. Development of more robust, recapitulative models will also be invaluable to investigate new and emerging therapeutics for the often devastating disease of systemic lymphangiomatosis.

UI MeSH Term Description Entries
D008202 Lymphangioma A benign tumor resulting from a congenital malformation of the lymphatic system. Lymphangioendothelioma is a type of lymphangioma in which endothelial cells are the dominant component. Lymphangioendothelioma,Lymphangioma, Cavernous,Endothelioma, Lymphatic,Cavernous Lymphangioma,Cavernous Lymphangiomas,Endotheliomas, Lymphatic,Lymphangioendotheliomas,Lymphangiomas,Lymphangiomas, Cavernous,Lymphatic Endothelioma,Lymphatic Endotheliomas
D008232 Lymphoproliferative Disorders Disorders characterized by proliferation of lymphoid tissue, general or unspecified. Duncan's Syndrome,X-Linked Lymphoproliferative Syndrome,Duncan Disease,Epstein-Barr Virus Infection, Familial Fatal,Epstein-Barr Virus-Induced Lymphoproliferative Disease In Males,Familial Fatal Epstein-Barr Infection,Immunodeficiency 5,Immunodeficiency, X-Linked Progressive Combined Variable,Lymphoproliferative Disease, X-Linked,Lymphoproliferative Syndrome, X-Linked, 1,Purtilo Syndrome,X-Linked Lymphoproliferative Disease,X-Linked Lymphoproliferative Disorder,Disease, Duncan,Disease, X-Linked Lymphoproliferative,Diseases, X-Linked Lymphoproliferative,Disorder, Lymphoproliferative,Disorder, X-Linked Lymphoproliferative,Disorders, Lymphoproliferative,Disorders, X-Linked Lymphoproliferative,Epstein Barr Virus Induced Lymphoproliferative Disease In Males,Epstein Barr Virus Infection, Familial Fatal,Familial Fatal Epstein Barr Infection,Immunodeficiency 5s,Immunodeficiency, X Linked Progressive Combined Variable,Lymphoproliferative Disease, X Linked,Lymphoproliferative Diseases, X-Linked,Lymphoproliferative Disorder,Lymphoproliferative Disorder, X-Linked,Lymphoproliferative Disorders, X-Linked,Lymphoproliferative Syndrome, X-Linked,Lymphoproliferative Syndromes, X-Linked,Purtilo Syndromes,Syndrome, Purtilo,Syndrome, X-Linked Lymphoproliferative,Syndromes, Purtilo,Syndromes, X-Linked Lymphoproliferative,X Linked Lymphoproliferative Disease,X Linked Lymphoproliferative Disorder,X Linked Lymphoproliferative Syndrome,X-Linked Lymphoproliferative Diseases,X-Linked Lymphoproliferative Disorders,X-Linked Lymphoproliferative Syndromes
D004195 Disease Models, Animal Naturally-occurring or experimentally-induced animal diseases with pathological processes analogous to human diseases. Animal Disease Model,Animal Disease Models,Disease Model, Animal
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000818 Animals Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA. Animal,Metazoa,Animalia
D015027 Zebrafish An exotic species of the family CYPRINIDAE, originally from Asia, that has been introduced in North America. Zebrafish is a model organism for drug assay and cancer research. Brachydanio rerio,Danio rerio,B. rerio,D. rerio,Zebra Fish,Zebra Fishes,Zebra danio,Zebrafishes,D. rerios,Fishes, Zebra,Zebra danios,danio, Zebra
D042583 Lymphangiogenesis The formation of LYMPHATIC VESSELS. Lymphangiogeneses
D042601 Lymphatic Vessels Tubular vessels that are involved in the transport of LYMPH and LYMPHOCYTES. Capillaries, Lymphatic,Lymphatic Capillaries,Lymphatic Venules,Venules, Lymphatic,Capillary, Lymphatic,Lymphatic Capillary,Lymphatic Venule,Lymphatic Vessel,Venule, Lymphatic,Vessel, Lymphatic,Vessels, Lymphatic
D051379 Mice The common name for the genus Mus. Mice, House,Mus,Mus musculus,Mice, Laboratory,Mouse,Mouse, House,Mouse, Laboratory,Mouse, Swiss,Mus domesticus,Mus musculus domesticus,Swiss Mice,House Mice,House Mouse,Laboratory Mice,Laboratory Mouse,Mice, Swiss,Swiss Mouse,domesticus, Mus musculus

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