http://purl.uniprot.org/citations/33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/33835027 | http://www.w3.org/2000/01/rdf-schema#comment | "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 thus 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."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.org/dc/terms/identifier | "doi:10.7554/elife.65158"xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Chang H.C."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Jiang X."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Sato T."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Ardehali H."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Sawicki K.T."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Shapiro J.S."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Geier J."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/author | "Senyei G."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/date | "2021"xsd:gYear |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/name | "Elife"xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/pages | "e65158"xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/title | "Augmenter of liver regeneration regulates cellular iron homeostasis by modulating mitochondrial transport of ATP-binding cassette B8."xsd:string |
http://purl.uniprot.org/citations/33835027 | http://purl.uniprot.org/core/volume | "10"xsd:string |
http://purl.uniprot.org/citations/33835027 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/33835027 |
http://purl.uniprot.org/citations/33835027 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/33835027 |
http://purl.uniprot.org/uniprot/#_B3KSN3-mappedCitation-33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33835027 |
http://purl.uniprot.org/uniprot/#_C9JTY4-mappedCitation-33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33835027 |
http://purl.uniprot.org/uniprot/#_H3BQQ4-mappedCitation-33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33835027 |
http://purl.uniprot.org/uniprot/#_L8EAK9-mappedCitation-33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33835027 |
http://purl.uniprot.org/uniprot/#_P55789-mappedCitation-33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33835027 |
http://purl.uniprot.org/uniprot/#_P56213-mappedCitation-33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33835027 |
http://purl.uniprot.org/uniprot/#_Q9CXJ4-mappedCitation-33835027 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33835027 |