Source:http://linkedlifedata.com/resource/pubmed/id/15769989
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
6
|
| pubmed:dateCreated |
2005-5-26
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| pubmed:abstractText |
Pheochromocytoma is a neuroendocrine tumor associated with a variety of genetic disorders, which include von Hippel-Lindau disease (VHL), multiple endocrine neoplasia type 2 (MEN 2), neurofibromatosis type 1, hereditary paraganglioma, and succinate dehydrogenase gene-related tumors. Previous studies of VHL-associated and MEN 2-associated pheochromocytomas suggest morphological, biochemical, and clinical differences exist among the tumors, but the process by which they develop remains unclear. Studies in other VHL-associated tumors suggest that VHL gene deficiency causes coexpression of erythropoietin (Epo) and its receptor (Epo-R), which facilitates tumor growth. The objective of this study was to understand the different process of tumorigenesis for VHL and MEN 2-associated pheochromocytomas. Ten pheochromocytomas (VHL patients n = 5, MEN 2 patients n = 5) were examined for the presence or absence of Epo and Epo-R using Western blot, immunohistochemistry, and RT-PCR analyses. Coexpression of Epo and Epo-R was found in all five VHL-associated pheochromocytomas; in contrast, expression of Epo-R, but not Epo, was documented in all five MEN 2-associated pheochromocytomas. Expression of Epo appears to be a result of VHL gene deficiency, possibly through activation of the hypoxia inducible factor-1 pathway, whereas Epo-R is an embryonal marker whose sustained expression in both VHL- and MEN 2-associated pheochromocytomas reflects an arrest or defect in development. These findings suggest an alternative process of tumorigenesis in VHL- and MEN 2-associated pheochromocytomas and implicate Epo as a clinical biomarker to differentiate these tumors.
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| pubmed:language |
eng
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| pubmed:journal | |
| pubmed:citationSubset |
AIM
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| pubmed:chemical | |
| pubmed:status |
MEDLINE
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| pubmed:month |
Jun
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| pubmed:issn |
0021-972X
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| pubmed:author |
pubmed-author:BrouwersFrederieke MFM,
pubmed-author:LinehanW MarstonWM,
pubmed-author:LubenskyIrina AIA,
pubmed-author:OldfieldEdward HEH,
pubmed-author:PacakKarelK,
pubmed-author:VogelTimothy W ATW,
pubmed-author:VortmeyerAlexander OAO,
pubmed-author:WaltherMcClellan MMM,
pubmed-author:WeilRobert JRJ,
pubmed-author:ZhuangZhengpingZ
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| pubmed:issnType |
Print
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| pubmed:volume |
90
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| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
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| pubmed:pagination |
3747-51
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| pubmed:dateRevised |
2007-11-15
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| pubmed:meshHeading |
pubmed-meshheading:15769989-Adrenal Gland Neoplasms,
pubmed-meshheading:15769989-Blotting, Western,
pubmed-meshheading:15769989-DNA Primers,
pubmed-meshheading:15769989-Erythropoietin,
pubmed-meshheading:15769989-Humans,
pubmed-meshheading:15769989-Immunohistochemistry,
pubmed-meshheading:15769989-Multiple Endocrine Neoplasia Type 2a,
pubmed-meshheading:15769989-Multiple Endocrine Neoplasia Type 2b,
pubmed-meshheading:15769989-Pheochromocytoma,
pubmed-meshheading:15769989-RNA, Messenger,
pubmed-meshheading:15769989-Receptors, Erythropoietin,
pubmed-meshheading:15769989-Reverse Transcriptase Polymerase Chain Reaction,
pubmed-meshheading:15769989-von Hippel-Lindau Disease
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| pubmed:year |
2005
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| pubmed:articleTitle |
Differential expression of erythropoietin and its receptor in von hippel-lindau-associated and multiple endocrine neoplasia type 2-associated pheochromocytomas.
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| pubmed:affiliation |
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 5D-37, Bethesda, Maryland 20892-1414, USA.
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| pubmed:publicationType |
Journal Article
|