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PDBsum entry 4myh
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Transport protein
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PDB id
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4myh
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References listed in PDB file
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Key reference
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Title
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Crystal structures of nucleotide-Free and glutathione-Bound mitochondrial abc transporter atm1.
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Authors
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V.Srinivasan,
A.J.Pierik,
R.Lill.
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Ref.
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Science, 2014,
343,
1137-1140.
[DOI no: ]
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PubMed id
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Abstract
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The yeast mitochondrial ABC transporter Atm1, in concert with glutathione,
functions in the export of a substrate required for cytosolic-nuclear
iron-sulfur protein biogenesis and cellular iron regulation. Defects in the
human ortholog ABCB7 cause the sideroblastic anemia XLSA/A. Here, we report the
crystal structures of free and glutathione-bound Atm1 in inward-facing, open
conformations at 3.06- and 3.38-angstrom resolution, respectively. The
glutathione binding site includes a residue mutated in XLSA/A and is located
close to the inner membrane surface in a large cavity. The two nucleotide-free
adenosine 5'-triphosphate binding domains do not interact yet are kept in close
vicinity through tight interaction of the two C-terminal α-helices of the Atm1
dimer. The resulting protein stabilization may be a common structural feature of
all ABC exporters.
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