Expression of angiogenic and vasculogenic proteins in the lung in alveolar capillary dysplasia/misalignment of pulmonary veins: An immunohistochemical study

Partha Sen, Tiyashi Choudhury, E. O.Brian Smith, Claire Langston*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

Abstract

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, universally fatal developmental disorder of the lung affecting both the parenchyma and the vasculature. Its cause remains incompletely understood; the occurrence of familial cases has suggested a genetic abnormality. While several candidate genes have been studied previously, the affected pathway(s) have not yet been fully defined. The expression patterns of 8 gene products (endothelial nitric oxide synthase-3, fetal liver kinase-1, hypoxia inducible factor 1a, Von Hippel Lindau protein, 3 vascular endothelial growth factors [VEGF147, VEGFC1, and VEGFA20], and activin receptor-like kinase 1), all known to have a role in vascular development in the lung, were studied in 13 ACD/MPV and 17 control lungs by immunohistochemistry to further address the underlying molecular abnormality. Expression was graded with regard to degree and extent for multiple components of the lung parenchyma and pulmonary vasculature for each antibody. Statistical analyses of the data using the Mann-Whitney test revealed only a few significant differences (P ≤ 0.05) in degree of expression between ACD/MPV and control lung samples and do not clearly implicate one of these genes in ACD/MPV.

Original languageEnglish (US)
Pages (from-to)354-361
Number of pages8
JournalPediatric and Developmental Pathology
Volume13
Issue number5
DOIs
StatePublished - Sep 1 2010

Keywords

  • Alveolar capillary dysplasia
  • Immunohistochemistry
  • Lung development
  • Misalignment of pulmonary veins

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

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