FOXF1 Antibody – Rabbit Polyclonal

FOXF1: Probable transcription activator for a number of lung- specific genes. Defects in FOXF1 are the cause of alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV). 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.

Protein type: Motility/polarity/chemotaxis; Transcription factor; DNA-binding

Chromosomal Location of Human Ortholog: 16q24

Cellular Component: transcription factor complex; nucleus

Molecular Function: RNA polymerase II transcription factor activity, enhancer binding; DNA binding; sequence-specific DNA binding

Biological Process: transcription from RNA polymerase II promoter; extracellular matrix organization and biogenesis; negative regulation of mast cell degranulation; positive regulation of transcription, DNA-dependent; heart development; establishment of epithelial cell polarity; negative regulation of transcription from RNA polymerase II promoter; lateral mesodermal cell differentiation; cell-cell adhesion; embryonic digestive tract morphogenesis; embryonic foregut morphogenesis; regulation of smooth muscle cell differentiation; positive regulation of mesenchymal cell proliferation; pancreas development; detection of wounding; vasculogenesis; blood vessel development; smoothened signaling pathway; somitogenesis; in utero embryonic development; respiratory tube development; morphogenesis of a branching structure; gut development; negative regulation of inflammatory response; midgut development; positive regulation of transcription from RNA polymerase II promoter; smooth muscle cell differentiation; embryonic ectodermal gut morphogenesis; determination of left/right symmetry; alveolus development; lung development; positive regulation of cell migration

Disease: Alveolar Capillary Dysplasia With Misalignment Of Pulmonary Veins