Characterization and classification of ATP-binding cassette transporter ABCA3 mutants in fatal surfactant deficiency.

@article{Matsumura2006CharacterizationAC,
  title={Characterization and classification of ATP-binding cassette transporter ABCA3 mutants in fatal surfactant deficiency.},
  author={Yoshihiro Matsumura and Nobuhiro Ban and Kazumitsu Ueda and Nobuya Inagaki},
  journal={The Journal of biological chemistry},
  year={2006},
  volume={281 45},
  pages={34503-14}
}
The ATP-binding cassette transporter ABCA3 is expressed predominantly at the limiting membrane of the lamellar bodies in lung alveolar type II cells. Recent study has shown that mutation of the ABCA3 gene causes fatal surfactant deficiency in newborns. In this study, we investigated in HEK293 cells the intracellular localization and N-glycosylation of the ABCA3 mutants so far identified in fatal surfactant deficiency patients. Green fluorescent protein-tagged L101P, L982P, L1553P, Q1591P, and… CONTINUE READING
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