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ABCA3 plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol (Klugbauer and Hofmann 1996, Yamano et al. 2001). Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921] (Shulenin et al. 2004).<br>The exact roles of ABCA2 (Vulevic et al. 2001, Kaminski et al. 2001), ABCA6 (Kaminski & Wenzel et al. 2001), ABCA9 (Piehler et al. 2002), ABCA10 (Wenzel et al. 2003) and ABCA12 (Annilo et al. 2002), candidates for ABC lipid transporter-related activities, need to be elucidated. Even thought cholesterol-responsiveness has been noted in experimental systems, contribution of these proteins in regulation or in active transport is not yet clear.
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