{"id":"https://openalex.org/W7221764310","doi":"https://doi.org/10.1016/j.jbc.2026.113648","title":"The malonyl-CoA synthetase ACSF3 is required for metabolic editing downstream of biotin carboxylation","display_name":"The malonyl-CoA synthetase ACSF3 is required for metabolic editing downstream of biotin carboxylation","publication_year":2026,"publication_date":"2026-10-14","ids":{"openalex":"https://openalex.org/W7221764310","doi":"https://doi.org/10.1016/j.jbc.2026.113648","pmid":"https://pubmed.ncbi.nlm.nih.gov/42874924"},"language":"en","primary_location":{"id":"doi:10.1016/j.jbc.2026.113648","doi":"https://doi.org/10.1016/j.jbc.2026.113648","is_oa":true,"landing_page_url":"https://doi.org/10.1016/j.jbc.2026.113648","pdf_url":null,"source":{"id":"https://openalex.org/S140251998","display_name":"Journal of Biological Chemistry","issn_l":"0021-9258","issn":["0021-9258","1067-8816","1083-351X"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Biological Chemistry","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://doi.org/10.1016/j.jbc.2026.113648","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5158734142","display_name":"Joseph Choi","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Joseph Choi","raw_affiliation_strings":["Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5116849575","display_name":"Jasmine Encarnacion","orcid":"https://orcid.org/0000-0002-6180-1309","observed_orcids":["https://orcid.org/0000-0002-6180-1309"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Jasmine Encarnacion","raw_affiliation_strings":["Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5158691885","display_name":"Daria Kurganova","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Daria Kurganova","raw_affiliation_strings":["Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5016274644","display_name":"Susanna Scafidi","orcid":"https://orcid.org/0000-0002-1229-8778","observed_orcids":["https://orcid.org/0000-0002-1229-8778"]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]},{"id":"https://openalex.org/I2799853436","display_name":"Johns Hopkins Medicine","ror":"https://ror.org/037zgn354","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I2799853436"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Susanna Scafidi","raw_affiliation_strings":["Anesthesiology and Critical Care Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Anesthesiology and Critical Care Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA","institution_ids":["https://openalex.org/I145311948","https://openalex.org/I2799853436"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5043042092","display_name":"Michael J. Wolfgang","orcid":"https://orcid.org/0000-0003-2349-8414","observed_orcids":["https://orcid.org/0000-0003-2349-8414"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Michael J. Wolfgang","raw_affiliation_strings":["Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA. Electronic address: mwolfga1@jhmi.edu"],"raw_orcid":"https://orcid.org/0000-0003-2349-8414","affiliations":[{"raw_affiliation_string":"Department of Physiology, Pharmacology & Therapeutics, Baltimore, Maryland USA. Electronic address: mwolfga1@jhmi.edu","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5043042092"],"corresponding_institution_ids":[],"apc_list":{"value":3430,"currency":"USD","value_usd":3430},"apc_paid":{"value":3430,"currency":"USD","value_usd":3430},"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.78457772,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"302","issue":"12","first_page":"113648","last_page":"113648"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.9868000149726868,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.9868000149726868,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T13127","display_name":"Biotin and Related Studies","score":0.01140000019222498,"subfield":{"id":"https://openalex.org/subfields/1307","display_name":"Cell Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11844","display_name":"Lipid metabolism and biosynthesis","score":0.0012000000569969416,"subfield":{"id":"https://openalex.org/subfields/1303","display_name":"Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/malonate","display_name":"malonate","score":0.9440000057220459},{"id":"https://openalex.org/keywords/methylmalonate","display_name":"methylmalonate","score":0.9990000128746033},{"id":"https://openalex.org/keywords/holocarboxylase-synthetase","display_name":"holocarboxylase synthetase","score":0.9120000004768372},{"id":"https://openalex.org/keywords/mitochondrial-metabolism","display_name":"mitochondrial metabolism","score":0.7350000143051147},{"id":"https://openalex.org/keywords/enzyme-redundancy","display_name":"enzyme redundancy","score":0.5820000171661377},{"id":"https://openalex.org/keywords/liver","display_name":"liver","score":0.3869999945163727},{"id":"https://openalex.org/keywords/inborn-errors-of-metabolism","display_name":"inborn errors of metabolism","score":0.7039999961853027}],"concepts":[{"id":"https://openalex.org/C2778204606","wikidata":"https://www.wikidata.org/wiki/Q181354","display_name":"Biotin","level":2,"score":0.8370000123977661},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.7074000239372253},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.6384999752044678},{"id":"https://openalex.org/C24428623","wikidata":"https://www.wikidata.org/wiki/Q901081","display_name":"Carboxylation","level":3,"score":0.5220999717712402},{"id":"https://openalex.org/C2776207758","wikidata":"https://www.wikidata.org/wiki/Q5303302","display_name":"Downstream (manufacturing)","level":2,"score":0.4207000136375427},{"id":"https://openalex.org/C2779664074","wikidata":"https://www.wikidata.org/wiki/Q3518405","display_name":"Terminal (telecommunication)","level":2,"score":0.3725000023841858},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.37130001187324524},{"id":"https://openalex.org/C140137476","wikidata":"https://www.wikidata.org/wiki/Q21114968","display_name":"Pyruvate carboxylase","level":3,"score":0.3142000138759613},{"id":"https://openalex.org/C62231903","wikidata":"https://www.wikidata.org/wiki/Q1057","display_name":"Metabolism","level":2,"score":0.29109999537467957},{"id":"https://openalex.org/C2781113356","wikidata":"https://www.wikidata.org/wiki/Q4864654","display_name":"Metabolic intermediate","level":3,"score":0.28369998931884766},{"id":"https://openalex.org/C6350086","wikidata":"https://www.wikidata.org/wiki/Q6822347","display_name":"Metabolic engineering","level":3,"score":0.26899999380111694},{"id":"https://openalex.org/C197957613","wikidata":"https://www.wikidata.org/wiki/Q407280","display_name":"Cofactor","level":3,"score":0.2621000111103058},{"id":"https://openalex.org/C2779281246","wikidata":"https://www.wikidata.org/wiki/Q172847","display_name":"Peptide","level":2,"score":0.26089999079704285},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.25839999318122864},{"id":"https://openalex.org/C2779318131","wikidata":"https://www.wikidata.org/wiki/Q21199067","display_name":"Acetyl-CoA carboxylase","level":4,"score":0.2549000084400177},{"id":"https://openalex.org/C204628709","wikidata":"https://www.wikidata.org/wiki/Q864854","display_name":"Biotinylation","level":2,"score":0.25429999828338623},{"id":"https://openalex.org/C125209161","wikidata":"https://www.wikidata.org/wiki/Q422816","display_name":"Enzyme activator","level":3,"score":0.2533000111579895}],"mesh":[],"locations_count":2,"locations":[{"id":"doi:10.1016/j.jbc.2026.113648","doi":"https://doi.org/10.1016/j.jbc.2026.113648","is_oa":true,"landing_page_url":"https://doi.org/10.1016/j.jbc.2026.113648","pdf_url":null,"source":{"id":"https://openalex.org/S140251998","display_name":"Journal of Biological Chemistry","issn_l":"0021-9258","issn":["0021-9258","1067-8816","1083-351X"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Biological Chemistry","raw_type":"journal-article"},{"id":"pmid:42874924","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/42874924","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"The Journal of biological chemistry","raw_type":"Journal Article"}],"best_oa_location":{"id":"doi:10.1016/j.jbc.2026.113648","doi":"https://doi.org/10.1016/j.jbc.2026.113648","is_oa":true,"landing_page_url":"https://doi.org/10.1016/j.jbc.2026.113648","pdf_url":null,"source":{"id":"https://openalex.org/S140251998","display_name":"Journal of Biological Chemistry","issn_l":"0021-9258","issn":["0021-9258","1067-8816","1083-351X"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Biological Chemistry","raw_type":"journal-article"},"sustainable_development_goals":[{"score":0.428600013256073,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":36,"referenced_works":["https://openalex.org/W129702359","https://openalex.org/W1969469789","https://openalex.org/W1982218364","https://openalex.org/W1991731026","https://openalex.org/W2006051121","https://openalex.org/W2020285011","https://openalex.org/W2042450476","https://openalex.org/W2061725130","https://openalex.org/W2068194665","https://openalex.org/W2092786020","https://openalex.org/W2107081864","https://openalex.org/W2111316498","https://openalex.org/W2123930041","https://openalex.org/W2127498339","https://openalex.org/W2135110132","https://openalex.org/W2154799239","https://openalex.org/W2158950938","https://openalex.org/W2160163805","https://openalex.org/W2319441209","https://openalex.org/W2442856557","https://openalex.org/W2610983028","https://openalex.org/W2738017965","https://openalex.org/W2740884150","https://openalex.org/W2763205993","https://openalex.org/W2772709248","https://openalex.org/W2896352304","https://openalex.org/W3049404831","https://openalex.org/W3216875646","https://openalex.org/W4225615922","https://openalex.org/W4281487571","https://openalex.org/W4307723480","https://openalex.org/W4323042675","https://openalex.org/W4388648107","https://openalex.org/W4389993180","https://openalex.org/W4405335700","https://openalex.org/W7160507138"],"related_works":["https://openalex.org/W2610983028","https://openalex.org/W2890634706","https://openalex.org/W2655885133","https://openalex.org/W2020285011","https://openalex.org/W3037009627","https://openalex.org/W2763205993","https://openalex.org/W7162197594","https://openalex.org/W2964860620","https://openalex.org/W7160659204","https://openalex.org/W2107081864"],"abstract_inverted_index":{"Acsf3,":[0],"a":[1,74,88,146],"mitochondrial":[2,49,154,162,180],"malonyl-CoA":[3,70],"synthetase,":[4],"is":[5],"mutated":[6],"in":[7,21,37,48,69,114,117,132,171,182],"humans":[8],"with":[9,145],"inborn":[10,62],"error":[11],"Combined":[12],"Malonic":[13],"and":[14,42,44,81,112,122,141,151,176],"Methylmalonic":[15],"Aciduria.":[16],"To":[17],"clarify":[18],"its":[19],"role":[20,168],"mammalian":[22],"metabolism,":[23,50],"we":[24],"generated":[25,86],"Acsf3":[26,35,64,150,170],"knockout":[27,148],"mice.":[28],"We":[29],"show":[30],"that":[31,93],"the":[32,52,60,98,102,129,137,157,167,183],"loss":[33,100],"of":[34,40,77,90,101,126,139,149,161,169,174,185],"results":[36,113],"high":[38],"concentrations":[39],"malonate":[41,140,175],"methylmalonate":[43,82,142,177],"exhibits":[45],"expected":[46],"defects":[47],"but":[51],"KO":[53,65],"mice":[54,66],"are":[55,83],"largely":[56],"healthy,":[57],"similar":[58],"to":[59,136,178],"human":[61],"error.":[63],"exhibit":[67],"redundancy":[68],"synthetase":[71],"activity,":[72],"suggesting":[73],"genetic":[75],"modifier":[76],"disease":[78],"severity.":[79],"Malonate":[80],"metabolic":[84,95,123,130,172],"poisons":[85],"as":[87],"by-product":[89],"biotin-dependent":[91,110],"carboxylation":[92,111],"require":[94],"editing.":[96],"Indeed,":[97],"liver-specific":[99],"biotin":[103],"ligase,":[104],"Holocarboxylase":[105],"Synthetase":[106],"(hlcs),":[107],"inhibits":[108],"all":[109],"broad":[115],"changes":[116],"liver":[118,134],"mRNA,":[119],"protein,":[120],"posttranslational,":[121],"regulation.":[124],"Loss":[125],"Hlcs":[127,152],"suppresses":[128],"deficits":[131],"Acsf3KO":[133],"due":[135],"inhibition":[138],"generation.":[143],"Mice":[144],"double":[147],"maintained":[153],"lipoylation,":[155],"demonstrating":[156],"highly":[158],"redundant":[159],"generation":[160],"malonyl-CoA.":[163],"These":[164],"data":[165],"define":[166],"editing":[173],"maintain":[179],"metabolism":[181],"face":[184],"endogenously":[186],"produced":[187],"anti-metabolites.":[188]},"counts_by_year":[],"updated_date":"2026-11-09T13:22:03.289127","created_date":"2026-10-15T00:00:00"}
