Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
2011-6-27
pubmed:abstractText
Pulmonary arterial hypertension (PAH) is a debilitating disease with a high mortality rate. A hallmark of PAH is plexiform lesions (PLs), complex vascular formations originating from remodeled pulmonary arteries. The development and significance of these lesions have been debated and are not yet fully understood. Some features of PLs resemble neoplastic disorders, and there is a striking resemblance to glomeruloid-like lesions (GLLs) in glioblastomas. To further elucidate PLs, we used in situ methods, such as (fluorescent) IHC staining, three-dimensional reconstruction, and laser microdissection, followed by mRNA expression analysis. We generated compartment-specific expression patterns in the lungs of 25 patients (11 with PAH associated with systemic shunts, 6 with idiopathic PAH, and 8 controls) and GLLs from 5 glioblastomas. PLs consisted of vascular channels lined by a continuously proliferating endothelium and backed by a uniform myogenic interstitium. They also showed up-regulation of remodeling-associated genes, such as HIF1a, TGF-?1, VEGF-?, VEGFR-1/-2, Ang-1, Tie-2, and THBS1, but also of cKIT and sprouting-associated markers, such as NOTCH and matrix metalloproteinases. The cellular composition and signaling seen in GLLs in neural neoplasms differed significantly from those in PLs. In conclusion, PLs show a distinct cellular composition and microenvironment, which contribute to the plexiform phenotype and set them apart from other processes of vascular remodeling in patients with PAH. Neoplastic models of angiogenesis seem to be of limited use in further study of plexiform vasculopathy.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
1525-2191
pubmed:author
pubmed:copyrightInfo
Copyright © 2011 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.
pubmed:issnType
Electronic
pubmed:volume
179
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
167-79
pubmed:dateRevised
2011-11-17
pubmed:meshHeading
pubmed-meshheading:21703400-Adult, pubmed-meshheading:21703400-Aged, pubmed-meshheading:21703400-Blotting, Western, pubmed-meshheading:21703400-Case-Control Studies, pubmed-meshheading:21703400-Endothelium, Vascular, pubmed-meshheading:21703400-Enzyme-Linked Immunosorbent Assay, pubmed-meshheading:21703400-Female, pubmed-meshheading:21703400-Flow Cytometry, pubmed-meshheading:21703400-Fluorescent Antibody Technique, pubmed-meshheading:21703400-Glioblastoma, pubmed-meshheading:21703400-Humans, pubmed-meshheading:21703400-Hypertension, Pulmonary, pubmed-meshheading:21703400-Immunoenzyme Techniques, pubmed-meshheading:21703400-Immunoprecipitation, pubmed-meshheading:21703400-Kidney Glomerulus, pubmed-meshheading:21703400-Male, pubmed-meshheading:21703400-Middle Aged, pubmed-meshheading:21703400-Neovascularization, Pathologic, pubmed-meshheading:21703400-RNA, Messenger, pubmed-meshheading:21703400-Reverse Transcriptase Polymerase Chain Reaction, pubmed-meshheading:21703400-Young Adult
pubmed:year
2011
pubmed:articleTitle
Plexiform lesions in pulmonary arterial hypertension composition, architecture, and microenvironment.
pubmed:affiliation
Institute of Pathology, Hannover Medical School, Hanover, Germany. jonigk.danny@mh-hannover.de
pubmed:publicationType
Journal Article, Comparative Study, Research Support, Non-U.S. Gov't