{"doi":"10.1101/2020.11.30.403295","title":"Augmenter of Liver Regeneration Regulates Cellular Iron Homeostasis by Modulating Mitochondrial Transport of ATP-Binding Cassette B8","abstract":"Abstract Chronic loss of Augmenter of Liver Regeneration (ALR) results in mitochondrial myopathy with cataracts, however, the mechanism for this disorder remains unclear. Here, we demonstrate that loss of ALR, a principal component of the MIA40/ALR protein import pathway, results in impaired cytosolic Fe/S cluster biogenesis in mammalian cells. Mechanistically, MIA40/ALR facilitates the mitochondrial import of ATP binding cassette (ABC)-B8, an inner mitochondrial membrane protein required for cytoplasmic Fe/S cluster maturation, through physical interaction with ABCB8. Downregulation of ALR impairs mitochondrial ABCB8 import, reduces cytoplasmic Fe/S cluster maturation, and increases cellular iron through the iron regulatory protein-iron response element system. Our finding provides a mechanistic link between MIA40/ALR import machinery and cytosolic Fe/S cluster maturation through the mitochondrial import of ABCB8, and offers a potential explanation for the pathology seen in patients with ALR mutations.","journal":"bioRxiv (Cold Spring Harbor Laboratory)","year":2020,"id":131730,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9558,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":117196,"name":"Jason Shapiro","orcid":"0000-0003-0880-3142","position":1,"is_corresponding":false},{"id":586470,"name":"Xinghang Jiang","orcid":null,"position":2,"is_corresponding":false},{"id":586471,"name":"Grant Senyei","orcid":null,"position":3,"is_corresponding":false},{"id":585919,"name":"Teruki Sato","orcid":"0000-0001-7860-9770","position":4,"is_corresponding":false},{"id":585920,"name":"Konrad Teodor Sawicki","orcid":"0000-0003-2124-0081","position":5,"is_corresponding":false},{"id":117198,"name":"Hossein Ardehali","orcid":"0000-0002-7662-0551","position":6,"is_corresponding":false},{"id":585918,"name":"Hsiang‐Chun Chang","orcid":"0000-0002-9201-4500","position":0,"is_corresponding":true}],"reference_count":41,"raw_metadata":null,"created_at":"2026-07-18T23:16:03.875886Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}