| Genetic analysis of congenital cystic adenomatoid malformation reveals a novel pulmonary gene: fatty acid binding protein-7 (brain type). | |
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MedLine Citation:
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PMID: 18391847 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The pathogenesis of congenital cystic adenomatoid malformation (CCAM) is unknown and its natural history is unpredictable. Fatty acid binding protein-7 (FABP-7) has been previously described in brain and breast development, but never before in the lung. We investigate gene expression in CCAM, and hypothesize that CCAM results from an aberration in the signaling pathway during lung development. Under IRB approval, tissue specimens of fetal CCAM, fetal control, postnatal CCAM, and postnatal control were examined and microarray analysis was performed. Candidate differentially expressed genes were selected with log-odds ratio (B) >0 and false discovery rate <0.05. Validation of differential expression was achieved at the RNA and protein levels. FABP-7 was underexpressed in fetal CCAM compared with fetal lung in both the microarray and by RT-PCR. Findings were duplicated by Western Blot analysis and immunohistochemistry. This is the first description of FABP-7 in the human lung. Decreased expression of FABP-7 in fetal CCAM compared with normal fetal lung at both the RNA and protein levels suggests FABP-7 may have a role in pulmonary development and in the pathogenesis of CCAM. |
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Authors:
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Amy J Wagner; Amber Stumbaugh; Zachary Tigue; Jess Edmondson; Agnes C Paquet; Diana L Farmer; Samuel Hawgood |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Pediatric research Volume: 64 ISSN: 0031-3998 ISO Abbreviation: Pediatr. Res. Publication Date: 2008 Jul |
Date Detail:
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Created Date: 2008-06-26 Completed Date: 2008-08-21 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0100714 Medline TA: Pediatr Res Country: United States |
Other Details:
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Languages: eng Pagination: 11-6 Citation Subset: IM |
Affiliation:
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Department of Surgery, University of California, San Francisco, San Francisco, California 94143, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Blotting, Western Carrier Proteins / analysis, genetics* Child Child, Preschool Cystic Adenomatoid Malformation of Lung, Congenital / embryology, genetics*, metabolism Down-Regulation Female Gene Expression Profiling / methods Gene Expression Regulation, Developmental* Genetic Predisposition to Disease Gestational Age Humans Immunohistochemistry Infant Infant, Newborn Lung / chemistry, embryology Male Oligonucleotide Array Sequence Analysis Reproducibility of Results Reverse Transcriptase Polymerase Chain Reaction Tumor Suppressor Proteins / analysis, genetics* |
| Grant Support | |
ID/Acronym/Agency:
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HL072301/HL/NHLBI NIH HHS; RR-00083/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Carrier Proteins; 0/FABP7 protein, human; 0/Tumor Suppressor Proteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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