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TitleCryo-EM structures of human ABCB7 reveal the molecular basis of mitochondrial matrix heme export.
Journal, issue, pagesCommun Biol, Year 2026
Publish dateMay 9, 2026
AuthorsSeulgi Ju / Seung Hun Choi / Hyeon You Lee / Mi Sun Jin /
PubMed AbstractATP-binding cassette transporter subfamily B member 7 (ABCB7) is a mitochondrial ATP-driven pump essential for cytosolic iron-sulfur (Fe-S) cluster biogenesis and cellular iron homeostasis. Mutations ...ATP-binding cassette transporter subfamily B member 7 (ABCB7) is a mitochondrial ATP-driven pump essential for cytosolic iron-sulfur (Fe-S) cluster biogenesis and cellular iron homeostasis. Mutations in ABCB7 are linked to X-linked sideroblastic anemia with ataxia (XLSA/A). Here, we demonstrate that the ATPase activity of ABCB7 is stimulated by iron and cobalt protoporphyrin IX (hemin and CoPP) in the presence of glutathione (GSH), highlighting an additional role for ABCB7 as a metalloporphyrin exporter. Using single-particle cryo-electron microscopy, we determine the structures of human ABCB7 in multiple functional states at resolutions of up to 2.3 Å. Our structures reveal a putative substrate-binding cavity that accommodates two stacked CoPP molecules conjugated by two GSH cysteine thiols. The conserved residue F426 controls substrate entrapment and release as a molecular gate. We further show that at high substrate concentrations excess CoPP easily partitions into the lipid bilayer, where the hydrophobic environment stabilizes the porphyrin macrocycle and limits the aggregation or redox reactivity that is prone to occur with free porphyrins in aqueous solution. Finally, our structure analyses rationalize the pathogenic effect of the E433K mutation associated with XLSA/A disease.
External linksCommun Biol / PubMed:42103889
MethodsEM (single particle)
Resolution2.33 - 3.01 Å
Structure data

69143

23pg

EMDB-69143, PDB-23pg:
Cryo-EM structure of human ABCB7 in complex with CoPP:GSH/ADPVO4
Method: EM (single particle) / Resolution: 2.87 Å

69144

23ph

EMDB-69144, PDB-23ph:
Cryo-EM structures of the human ABCB7 in the apo state
Method: EM (single particle) / Resolution: 3.01 Å

69145

23pi

EMDB-69145, PDB-23pi:
Cryo-EM structure of the human ABCB7 in occluded state
Method: EM (single particle) / Resolution: 2.33 Å

Chemicals

ChemComp-COH:
PROTOPORPHYRIN IX CONTAINING CO

ChemComp-CL:
Unknown entry

ChemComp-GSH:
GLUTATHIONE

ChemComp-MG:
Unknown entry

ChemComp-ADP:
ADENOSINE-5'-DIPHOSPHATE / ADP, energy-carrying molecule*YM

ChemComp-VO4:
VANADATE ION

ChemComp-ATP:
ADENOSINE-5'-TRIPHOSPHATE / ATP, energy-carrying molecule*YM

ChemComp-HOH:
WATER

Source
  • homo sapiens (human)
KeywordsMEMBRANE PROTEIN / ABCB7 / heme exporter / X-linked sideroblastic anemia with ataxia / cobalt protoporphyrin IX / iron-sulfur (Fe-S) cluster / glutathione

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