title: Bactericidal/Permeability-increasing protein fold-containing family member A1 in airway host protection and respiratory disease.
abstract: Bactericidal/permeability-increasing protein fold-containing family member A1 (BPIFA1), formerly known as SPLUNC1, is one of the most abundant proteins in respiratory secretions and has been identified with increasing frequency in studies of pulmonary disease. Its expression is largely restricted to the respiratory tract, being highly concentrated in the upper airways and proximal trachea. BPIFA1 is highly responsive to airborne pathogens, allergens, and irritants. BPIFA1 actively participates in host protection through antimicrobial, surfactant, airway surface liquid regulation, and immunomodulatory properties. Its expression is modulated in multiple lung diseases, including cystic fibrosis, chronic obstructive pulmonary disease, respiratory malignancies, and idiopathic pulmonary fibrosis. However, the role of BPIFA1 in pulmonary pathogenesis remains to be elucidated. This review highlights the versatile properties of BPIFA1 in antimicrobial protection and its roles as a sensor of environmental exposure and regulator of immune cell function. A greater understanding of the contribution of BPIFA1 to disease pathogenesis and activity may clarify if BPIFA1 is a biomarker and potential drug target in pulmonary disease.
authors.affiliation: Section of Pulmonary, Critical Care and Sleep Medicine, Yale University School of Medicine, New Haven, Connecticut.
authors.forename: Clemente J
authors.initials: CJ
authors.lastname: Britto
authors.forename: Lauren
authors.initials: L
authors.lastname: Cohn
mesh_terms: D000818:Animals; D005786:Gene Expression Regulation; D006023:Glycoproteins / Q000235:genetics / Q000378:metabolism*; D054884:Host-Pathogen Interactions; D006801:Humans; D007113:Immunity, Innate; D018836:Inflammation Mediators / Q000378:metabolism; D008171:Lung Diseases / Q000235:genetics / Q000276:immunology / Q000382:microbiology* / Q000821:virology; D010750:Phosphoproteins / Q000235:genetics / Q000378:metabolism*; D020545:Respiratory Mucosa / Q000276:immunology / Q000378:metabolism* / Q000382:microbiology / Q000821:virology; D015398:Signal Transduction
chemical_list: C485599:BPIFA1 protein, human; D006023:Glycoproteins; D018836:Inflammation Mediators; D010750:Phosphoproteins
keywords: BPIFA1; SPLUNC1; airway epithelium; host protection; inflammation