Anti-FOXF1抗体(ab23194)

Probable transcription activator for a number of lung-specific genes.

Lung and placenta.

Defects 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.

Contains 1 fork-head DNA-binding domain.

Activation domains C-terminal of (and distinct from) the forkhead domains are necessary for transcriptional activation.

Nucleus.