Article
Functional and trafficking defects in ATP binding cassette A3 mutants associated with respiratory distress syndrome.
The Journal of biological chemistry - 7 Apr 2006
Cheong Naeun, Madesh Muniswamy, Gonzales Linda W, Zhao Ming, Yu Kevin, Ballard Philip L, Shuman Henry
Abstract excerpt
Members of the ATP binding cassette (ABC) protein superfamily actively transport a wide range of substrates across cell and intracellular membranes. Mutations in ABCA3, a member of the ABCA subfamily with unknown function, lead to fatal respiratory distress syndrome (RDS) in the newborn. Using cultured human lung cells, we found that recombinant wild-type hABCA3 localized to membranes of both lysosomes and...
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