Logo image
Novel FOXF1 mutations in sporadic and familial cases of Alveolar Capillary Dysplasia with Misaligned Pulmonary Veins imply a role for its DNA binding domain
Journal article   Open access   Peer reviewed

Novel FOXF1 mutations in sporadic and familial cases of Alveolar Capillary Dysplasia with Misaligned Pulmonary Veins imply a role for its DNA binding domain

Partha Sen, Yaping Yang, Colby Navarro, Iris Silva, Przemyslaw Szafranski, Katarzyna E Kolodziejska, Avinash V Dharmadhikari, Hasnaa Mostafa, Harry Kozakewich, Debra Kearney, …
Human mutation, Vol.34(6), pp.801-811
06/2013
DOI: 10.1002/humu.22313
PMCID: PMC3663886
PMID: 23505205
url
https://doi.org/10.1002/humu.22313View
Published (Version of record) Open Access

Abstract

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare and lethal developmental disorder of the lung defined by a constellation of characteristic histopathological features. Non-pulmonary anomalies involving organs of gastrointestinal, cardiovascular, and genitourinary systems have been identified in approximately 80% of patients with ACD/MPV. We have collected DNA and pathological samples from more than 90 infants with ACD/MPV and their family members. Since the publication of our initial report of four point mutations and ten deletions, we have identified an additional thirty eight novel nonsynonymous mutations of
Angiogenesis ACD MPV FOXF1 Lung Imprinting Development

Details

Metrics

Logo image