Linked Life Data

17102089

Source:http://linkedlifedata.com/resource/pubmed/id/17102089

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2006-11-14
pubmed:abstractText
Pheochromocytomas and paragangliomas are neural-crest-derived tumors that arise from mutations in RET, VHL, NF1, and in the genes-encoding succinate dehydrogenase (SDH) subunits B (SDHB), C (SDHC), and D (SDHD). Despite their genetic diversity, these tumors cannot be clearly distinguished on the basis of their primary mutation. We recently identified two major transcriptional programs embedded within familial and sporadic pheochromocytomas and paragangliomas using global expression profiling. This review will summarize the major results of these studies and discuss their implications. The transcription data revealed that: (a) tumors with mutations in VHL, SDHB, and SDHD genes share a transcription signature of hypoxia, angiogenesis, and oxidoreductase imbalance; (b) SDHB protein is suppressed in tumors with mutations in SDHB and SDHD, and also in a subset of tumors with VHL mutations; and (c) HIF1alpha is involved in the SDHB downregulation observed in these tumors. These results are consistent with the existence of a close interconnection between the VHL and SDH pathways mediated predominantly by hypoxia and oxidoreductase signals. It further suggests that low SDHB levels indicative of impaired mitochondrial complex II function may be a shared element of these pheochromocytomas. SDHB may thus constitute a marker for tumors with abnormal hypoxic profile.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0077-8923
pubmed:author
pubmed:issnType
Print
pubmed:volume
1073
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
208-20
pubmed:meshHeading
pubmed:year
2006
pubmed:articleTitle
Transcription association of VHL and SDH mutations link hypoxia and oxidoreductase signals in pheochromocytomas.
pubmed:affiliation
Department of Medicine, University of Texas Health Science Center, 7703 Floyd Curl Drive, Room No. 5053-R3, MC 7880, San Antonio-TX 8229-3900, USA. dahia@uthscsa.edu
pubmed:publicationType
Journal Article, Review, Research Support, Non-U.S. Gov't