The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 2.5 µg/ml. Detects a band of approximately 45 kDa (predicted molecular weight: 40 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
FunctionProbable transcription activator for a number of lung-specific genes.
Tissue specificityLung and placenta.
Involvement in diseaseDefects in FOXF1 are the cause of alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) [MIM:265380]. ACDMPV is a rare malformation due to abnormal development of the capillary vascular system in the lungs. Histologically, it is characterized by failure of formation and ingrowth of alveolar capillaries, medial muscular thickening of small pulmonary arterioles with muscularization of the intraacinar arterioles, thickened alveolar walls, and anomalously situated pulmonary veins running alongside pulmonary arterioles and sharing the same adventitial sheath. Less common features include a reduced number of alveoli and a patchy distribution of the histopathologic changes. Affected infants present with respiratory distress and the disease is fatal within the newborn period. Additional features include multiple congenital anomalies affecting the cardiovascular, gastrointestinal, genitourinary, and musculoskeletal systems, as well as disruption of the normal right-left asymmetry of intrathoracic or intraabdominal organs. ACDMPV is a rare cause of persistent pulmonary hypertension of the newborn, an abnormal physiologic state caused by failure of transition of the pulmonary circulation from the high pulmonary vascular resistance of the fetus to the low pulmonary vascular resistance of the newborn.
Bonuccelli G et al. Caveolin-1 (P132L), a common breast cancer mutation, confers mammary cell invasiveness and defines a novel stem cell/metastasis-associated gene signature. Am J Pathol174:1650-62 (2009).
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