Abstract
The ABCA3 gene, of the ABCA subclass of ATP-binding cassette (ABC) transporters, is expressed exclusively in lung. We report here the cloning, molecular characterization, and distribution of human ABCA3 in the lung. Immunoblot analysis using the specific antibody reveals a 150-kDa protein in the crude membrane fraction of human lung. Immunohistochemical analyses of alveoli show that ABCA3 is expressed only in the type II cells expressing surfactant protein A. At the ultrastructural level, ABCA3 immunoreactivity was detected mostly at the limiting membrane of the lamellar bodies. Since members of the ABCA transporter family are known to be involved in transmembrane transport of endogenous lipids, our findings suggest that ABCA3 plays an important role in the formation of pulmonary surfactant in type II cells.
Publication types
-
Research Support, Non-U.S. Gov't
MeSH terms
-
ATP-Binding Cassette Transporters / analysis
-
ATP-Binding Cassette Transporters / genetics*
-
ATP-Binding Cassette Transporters / immunology
-
ATP-Binding Cassette Transporters / metabolism*
-
Antibodies / immunology
-
Blotting, Western
-
Cell Line
-
Cloning, Molecular
-
Humans
-
Immunohistochemistry
-
Microscopy, Confocal
-
Molecular Sequence Data
-
Organ Specificity
-
Proteolipids / metabolism
-
Pulmonary Alveoli / cytology*
-
Pulmonary Alveoli / metabolism*
-
Pulmonary Alveoli / ultrastructure
-
Pulmonary Surfactant-Associated Proteins
-
Pulmonary Surfactants / biosynthesis
-
Pulmonary Surfactants / metabolism
Substances
-
ABCA3 protein, human
-
ATP-Binding Cassette Transporters
-
Antibodies
-
Proteolipids
-
Pulmonary Surfactant-Associated Proteins
-
Pulmonary Surfactants
Associated data
-
GENBANK/AA638829
-
GENBANK/AB070929
-
GENBANK/AW900257