{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T14:55:48Z","timestamp":1790780148117,"version":"4.1.0"},"reference-count":90,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["2022R1A2C2091397"],"award-info":[{"award-number":["2022R1A2C2091397"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["RS\u20102023\u201000218623"],"award-info":[{"award-number":["RS\u20102023\u201000218623"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["advanced.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Advanced Science"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Galactokinase 1 (GALK1) is a metabolic kinase that phosphorylates galactose. Mutations in GALK1 cause type II galactosemia, which manifests as cataracts and extra\u2010ocular symptoms. The mechanisms underlying these symptoms are not fully understood. In this study, we explored a novel function of GALK1 beyond galactose phosphorylation. GALK1 is ubiquitously expressed across human tissues, regardless of developmental stage. GALK1 phosphorylates TIMM13 at the Y73 residue. The phosphorylated Y73 stabilizes TIMM13 by electrostatically interacting with two cationic residues, thereby enhancing its affinity for Zn\n                    <jats:sup>2<\/jats:sup>\n                    <jats:sup>+<\/jats:sup>\n                    . This structural change ensures the mitochondrial translocation of TIMM13 in the correct conformation. Disruption of Y73 phosphorylation impairs mitochondrial bioenergetics and dissipates the mitochondrial membrane potential. Furthermore, galactose competitively inhibits GALK1\u2010mediated phosphorylation, suggesting that mitochondrial activity is modulated by carbohydrate availability. Galactosemia\u2010associated GALK1 mutations showed compromised activity in TIMM13 phosphorylation, subsequently repressing mitochondrial respiration. Collectively, the GALK1\u2010TIMM13 axis appears to provide a potential link between galactose metabolism and mitochondrial respiration.\n                  <\/jats:p>","DOI":"10.1002\/advs.77996","type":"journal-article","created":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T14:04:37Z","timestamp":1790777077000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Galactokinase 1 Positively Regulates Mitochondrial Respiration by Phosphorylating TIMM13 as a Protein Kinase"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3575-1309","authenticated-orcid":false,"given":"Chang Woo","family":"Ko","sequence":"first","affiliation":[{"name":"Department of Biomedical Sciences College of Medicine Seoul National University  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5493-5026","authenticated-orcid":false,"given":"Dongyoon","family":"Shin","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences College of Medicine Seoul National University  Seoul Republic of Korea"},{"name":"Department of Medical Science School of Medicine CHA University  Seongnam Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1678-5968","authenticated-orcid":false,"given":"Junho","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Medical Science School of Medicine CHA University  Seongnam Republic of Korea"},{"name":"Department of Medical Science Research Institute for Basic Medical Science School of Medicine CHA University  Seongnam Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4610-4521","authenticated-orcid":false,"given":"June","family":"Huh","sequence":"additional","affiliation":[{"name":"Department of Chemical and Biological Engineering Korea University  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8881-0662","authenticated-orcid":false,"given":"Youngsoo","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences College of Medicine Seoul National University  Seoul Republic of Korea"},{"name":"Department of Medical Science School of Medicine CHA University  Seongnam Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4676-5191","authenticated-orcid":false,"given":"Jong\u2010Wan","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences College of Medicine Seoul National University  Seoul Republic of Korea"},{"name":"Department of Pharmacology College of Medicine Seoul National University  Seoul Republic of Korea"},{"name":"Ischemic\/Hypoxic Disease Institute Seoul National Medical Research Center Seoul National University  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,30]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018\u2010004\u20104160\u20106"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1022875629436"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545\u2010010\u20109270\u20108"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2018.01.006"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41568\u2010025\u201000800\u20103"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cmet.2022.08.002"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/advs.202501894"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467\u2010020\u201019263\u20101"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncb3338"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1126\/sciadv.aav4570"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2008.07.069"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.5.477"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.150.6.1271"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.15252\/embr.202256430"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41419\u2010023\u201005910\u20100"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432\u20101033.2003.03538.x"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/0076\u20106879(75)42091\u20102"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1086\/319512"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471\u20102091\u20104\u201016"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbapap.2016.10.012"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1126\/scisignal.2001570"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2005.08.064"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2018.12.092"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M412916200"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M115.643213"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/nsb780"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586\u2010021\u201003819\u20102"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms140816685"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdg421"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M808691200"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1021\/cn200074d"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1039\/C4MB00332B"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.27097"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1007\/s100380050182"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1093\/gigascience\/giae014"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkaa1034"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1242\/dmm.022988"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/mog2.70024"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1203\/PDR.0b013e3181761888"},{"key":"e_1_2_10_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2018.03.008"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586\u2010022\u201005574\u20104"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.abn4705"},{"key":"e_1_2_10_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.stem.2016.04.015"},{"key":"e_1_2_10_45_1","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.13374"},{"key":"e_1_2_10_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2014.03.032"},{"key":"e_1_2_10_47_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.091777"},{"key":"e_1_2_10_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cmet.2013.11.020"},{"key":"e_1_2_10_49_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.20\u201016\u201006048.2000"},{"key":"e_1_2_10_50_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M311017200"},{"key":"e_1_2_10_51_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1237797100"},{"key":"e_1_2_10_52_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450\u2010200205000\u201000010"},{"key":"e_1_2_10_53_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature19057"},{"key":"e_1_2_10_54_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586\u2010020\u20102308\u20107"},{"key":"e_1_2_10_55_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586\u2010021\u201003205\u2010y"},{"key":"e_1_2_10_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2007.04.005"},{"key":"e_1_2_10_57_1","doi-asserted-by":"publisher","DOI":"10.1021\/acs.jproteome.3c00632"},{"key":"e_1_2_10_58_1","doi-asserted-by":"publisher","DOI":"10.1086\/302611"},{"key":"e_1_2_10_59_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098\u20101004(200005)15:5<447::AID\u2010HUMU6>3.0.CO;2\u2010M"},{"key":"e_1_2_10_60_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkt1113"},{"key":"e_1_2_10_61_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450\u2010200205000\u201000009"},{"key":"e_1_2_10_62_1","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.17\u201021469"},{"key":"e_1_2_10_63_1","first-page":"682","article-title":"Autosomal Recessive Congenital Cataract in Consanguineous Pakistani Families Is Associated with Mutations in GALK1","volume":"16","author":"Yasmeen A.","year":"2010","journal-title":"Molecular Vision"},{"key":"e_1_2_10_64_1","doi-asserted-by":"publisher","DOI":"10.1038\/nprot.2009.154"},{"key":"e_1_2_10_65_1","doi-asserted-by":"publisher","DOI":"10.1016\/1044\u20100305(94)80016\u20102"},{"key":"e_1_2_10_66_1","doi-asserted-by":"crossref","first-page":"D609","DOI":"10.1093\/nar\/gkae1010","article-title":"UniProt: the Universal Protein Knowledgebase in 2025","volume":"53","author":"UniProt C.","year":"2025","journal-title":"Nucleic Acids Research"},{"key":"e_1_2_10_67_1","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1038\/ng.2653","article-title":"The Genotype\u2010Tissue Expression (GTEx) Project","volume":"45","author":"Consortium G. T.","year":"2013","journal-title":"Nature Genetics"},{"key":"e_1_2_10_68_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159\u20108290.CD\u201012\u20100095"},{"key":"e_1_2_10_69_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2764"},{"key":"e_1_2_10_70_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0506580102"},{"key":"e_1_2_10_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cels.2015.12.004"},{"key":"e_1_2_10_72_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature10523"},{"key":"e_1_2_10_73_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0258554"},{"key":"e_1_2_10_74_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0506\u2010500"},{"key":"e_1_2_10_75_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature08460"},{"key":"e_1_2_10_76_1","first-page":"507","article-title":"Frequency Distribution of the Values of the Correlation Coefficient in Samples From an Indefinitely Large Population","volume":"10","author":"Fisher R. A.","year":"1914","journal-title":"Biometrika"},{"key":"e_1_2_10_77_1","volume-title":"Methods of Meta\u2010analysis: Correcting Error and Bias in Research Findings","author":"Hunter J. E.","year":"2015"},{"key":"e_1_2_10_78_1","doi-asserted-by":"publisher","DOI":"10.1002\/jemt.20215"},{"key":"e_1_2_10_79_1","doi-asserted-by":"publisher","DOI":"10.1002\/jemt.20376"},{"key":"e_1_2_10_80_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkab1061"},{"key":"e_1_2_10_81_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586\u2010024\u201007487\u2010w"},{"key":"e_1_2_10_82_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcc.23354"},{"key":"e_1_2_10_83_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btu097"},{"key":"e_1_2_10_84_1","doi-asserted-by":"publisher","DOI":"10.1103\/PhysRevLett.78.2690"},{"key":"e_1_2_10_85_1","doi-asserted-by":"publisher","DOI":"10.1038\/258598a0"},{"key":"e_1_2_10_86_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pep.2011.04.022"},{"key":"e_1_2_10_87_1","doi-asserted-by":"publisher","DOI":"10.1002\/jssc.200401833"},{"key":"e_1_2_10_88_1","doi-asserted-by":"publisher","DOI":"10.1021\/cr0404390"},{"key":"e_1_2_10_89_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006\u20103495(01)76183\u20108"},{"key":"e_1_2_10_90_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102818.1993.tb03313.x"},{"key":"e_1_2_10_91_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.143.7.1883"}],"container-title":["Advanced Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/advs.77996","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/advs.77996","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/advs.77996","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T14:04:39Z","timestamp":1790777079000},"score":1,"resource":{"primary":{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/10.1002\/advs.77996"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,30]]},"references-count":90,"alternative-id":["10.1002\/advs.77996"],"URL":"https:\/\/doi.org\/10.1002\/advs.77996","archive":["Portico"],"relation":{},"ISSN":["2198-3844","2198-3844"],"issn-type":[{"value":"2198-3844","type":"print"},{"value":"2198-3844","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,30]]},"assertion":[{"value":"2026-04-23","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-18","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-30","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e77996"}}