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ABSTRACT:
SUBMITTER: Ellinghaus TL
PROVIDER: S-EPMC8694623 | biostudies-literature | 2021 Dec
REPOSITORIES: biostudies-literature
Ellinghaus Thomas L TL Marcellino Thomas T Srinivasan Vasundara V Lill Roland R Kühlbrandt Werner W
Science advances 20211222 52
The mitochondrial inner membrane ABC transporter Atm1 exports an unknown substrate to the cytosol for iron-sulfur protein biogenesis, cellular iron regulation, and tRNA thio-modification. Mutations in the human relative ABCB7 cause the iron storage disease XLSA/A. We determined 3D structures of two complementary states of Atm1 in lipid nanodiscs by electron cryo-microscopy at 2.9- to 3.4-Å resolution. The inward-open structure resembled the known crystal structure of nucleotide-free apo-Atm1 clo ...[more]