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Article

Cryo-EM structures of human ABCB7 reveal the molecular basis of mitochondrial matrix heme export.

Communications biology - 9 May 2026

Ju Seulgi, Choi Seung Hun, Lee Hyeon You, Jin Mi Sun

Abstract excerpt

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...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 42103889
DOI: 10.1038/s42003-026-10223-x
PMCID: PMC13376393

Genes

ABCB7

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

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