ID H7C2W1_HUMAN Unreviewed; 129 AA. AC H7C2W1; DT 18-APR-2012, integrated into UniProtKB/TrEMBL. DT 18-APR-2012, sequence version 1. DT 27-MAR-2024, entry version 74. DE RecName: Full=D-beta-hydroxybutyrate dehydrogenase, mitochondrial {ECO:0000256|ARBA:ARBA00039904}; DE EC=1.1.1.30 {ECO:0000256|ARBA:ARBA00038959}; DE AltName: Full=3-hydroxybutyrate dehydrogenase {ECO:0000256|ARBA:ARBA00041774}; DE Flags: Fragment; GN Name=BDH1 {ECO:0000313|Ensembl:ENSP00000408016.1}; OS Homo sapiens (Human). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; OC Homo. OX NCBI_TaxID=9606 {ECO:0000313|Ensembl:ENSP00000408016.1, ECO:0000313|Proteomes:UP000005640}; RN [1] {ECO:0000313|Ensembl:ENSP00000408016.1, ECO:0000313|Proteomes:UP000005640} RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. RX PubMed=16641997; DOI=10.1038/nature04728; RA Muzny D.M., Scherer S.E., Kaul R., Wang J., Yu J., Sudbrak R., Buhay C.J., RA Chen R., Cree A., Ding Y., Dugan-Rocha S., Gill R., Gunaratne P., RA Harris R.A., Hawes A.C., Hernandez J., Hodgson A.V., Hume J., Jackson A., RA Khan Z.M., Kovar-Smith C., Lewis L.R., Lozado R.J., Metzker M.L., RA Milosavljevic A., Miner G.R., Morgan M.B., Nazareth L.V., Scott G., RA Sodergren E., Song X.Z., Steffen D., Wei S., Wheeler D.A., Wright M.W., RA Worley K.C., Yuan Y., Zhang Z., Adams C.Q., Ansari-Lari M.A., Ayele M., RA Brown M.J., Chen G., Chen Z., Clendenning J., Clerc-Blankenburg K.P., RA Chen R., Chen Z., Davis C., Delgado O., Dinh H.H., Dong W., Draper H., RA Ernst S., Fu G., Gonzalez-Garay M.L., Garcia D.K., Gillett W., Gu J., RA Hao B., Haugen E., Havlak P., He X., Hennig S., Hu S., Huang W., RA Jackson L.R., Jacob L.S., Kelly S.H., Kube M., Levy R., Li Z., Liu B., RA Liu J., Liu W., Lu J., Maheshwari M., Nguyen B.V., Okwuonu G.O., RA Palmeiri A., Pasternak S., Perez L.M., Phelps K.A., Plopper F.J., Qiang B., RA Raymond C., Rodriguez R., Saenphimmachak C., Santibanez J., Shen H., RA Shen Y., Subramanian S., Tabor P.E., Verduzco D., Waldron L., Wang J., RA Wang J., Wang Q., Williams G.A., Wong G.K., Yao Z., Zhang J., Zhang X., RA Zhao G., Zhou J., Zhou Y., Nelson D., Lehrach H., Reinhardt R., RA Naylor S.L., Yang H., Olson M., Weinstock G., Gibbs R.A.; RT "The DNA sequence, annotation and analysis of human chromosome 3."; RL Nature 440:1194-1198(2006). RN [2] {ECO:0007829|PubMed:24275569} RP IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]. RX PubMed=24275569; DOI=10.1016/j.jprot.2013.11.014; RA Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., RA Ye M., Zou H.; RT "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver RT phosphoproteome."; RL J. Proteomics 96:253-262(2014). RN [3] {ECO:0007829|PubMed:25944712} RP IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]. RX PubMed=25944712; RA Vaca Jacome A.S., Rabilloud T., Schaeffer-Reiss C., Rompais M., Ayoub D., RA Lane L., Bairoch A., Van Dorsselaer A., Carapito C.; RT "N-terminome analysis of the human mitochondrial proteome."; RL Proteomics 15:2519-2524(2015). RN [4] {ECO:0000313|Ensembl:ENSP00000408016.1} RP IDENTIFICATION. RG Ensembl; RL Submitted (NOV-2023) to UniProtKB. CC -!- CATALYTIC ACTIVITY: CC Reaction=(R)-3-hydroxybutanoate + NAD(+) = acetoacetate + H(+) + NADH; CC Xref=Rhea:RHEA:20521, ChEBI:CHEBI:10983, ChEBI:CHEBI:13705, CC ChEBI:CHEBI:15378, ChEBI:CHEBI:57540, ChEBI:CHEBI:57945; EC=1.1.1.30; CC Evidence={ECO:0000256|ARBA:ARBA00035857}; CC -!- SUBUNIT: Homotetramer. {ECO:0000256|ARBA:ARBA00011881}. CC -!- SUBCELLULAR LOCATION: Membrane {ECO:0000256|ARBA:ARBA00004370}. CC Mitochondrion inner membrane {ECO:0000256|ARBA:ARBA00004273}. CC -!- SIMILARITY: Belongs to the short-chain dehydrogenases/reductases (SDR) CC family. {ECO:0000256|ARBA:ARBA00006484}. CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; AC128709; -; NOT_ANNOTATED_CDS; Genomic_DNA. DR EMBL; AC132008; -; NOT_ANNOTATED_CDS; Genomic_DNA. DR EPD; H7C2W1; -. DR MassIVE; H7C2W1; -. DR MaxQB; H7C2W1; -. DR PeptideAtlas; H7C2W1; -. DR ProteomicsDB; 45122; -. DR Antibodypedia; 33961; 293 antibodies from 30 providers. DR Ensembl; ENST00000455876.1; ENSP00000408016.1; ENSG00000161267.12. DR UCSC; uc062sep.1; human. DR HGNC; HGNC:1027; BDH1. DR VEuPathDB; HostDB:ENSG00000161267; -. DR GeneTree; ENSGT00940000156929; -. DR HOGENOM; CLU_010194_40_0_1; -. DR ChiTaRS; BDH1; human. DR Proteomes; UP000005640; Chromosome 3. DR Bgee; ENSG00000161267; Expressed in right lobe of liver and 102 other cell types or tissues. DR ExpressionAtlas; H7C2W1; baseline and differential. DR GO; GO:0005743; C:mitochondrial inner membrane; IEA:UniProtKB-SubCell. DR GO; GO:0016616; F:oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor; IEA:UniProt. DR GO; GO:0006629; P:lipid metabolic process; IEA:UniProtKB-KW. DR Gene3D; 3.40.50.720; NAD(P)-binding Rossmann-like Domain; 1. DR InterPro; IPR036291; NAD(P)-bd_dom_sf. DR InterPro; IPR020904; Sc_DH/Rdtase_CS. DR InterPro; IPR002347; SDR_fam. DR PANTHER; PTHR43313:SF25; D-BETA-HYDROXYBUTYRATE DEHYDROGENASE, MITOCHONDRIAL; 1. DR PANTHER; PTHR43313; SHORT-CHAIN DEHYDROGENASE/REDUCTASE FAMILY 9C; 1. DR Pfam; PF00106; adh_short; 1. DR SUPFAM; SSF51735; NAD(P)-binding Rossmann-fold domains; 1. DR PROSITE; PS00061; ADH_SHORT; 1. PE 1: Evidence at protein level; KW Allosteric enzyme {ECO:0000256|ARBA:ARBA00022533}; KW Lipid metabolism {ECO:0000256|ARBA:ARBA00023098}; KW Membrane {ECO:0000256|ARBA:ARBA00023136}; KW Mitochondrion {ECO:0000256|ARBA:ARBA00023128}; KW Mitochondrion inner membrane {ECO:0000256|ARBA:ARBA00022792}; KW Oxidoreductase {ECO:0000256|ARBA:ARBA00023002}; KW Proteomics identification {ECO:0007829|EPD:H7C2W1, KW ECO:0007829|MaxQB:H7C2W1}; KW Reference proteome {ECO:0000313|Proteomes:UP000005640}. FT NON_TER 1 FT /evidence="ECO:0000313|Ensembl:ENSP00000408016.1" FT NON_TER 129 FT /evidence="ECO:0000313|Ensembl:ENSP00000408016.1" SQ SEQUENCE 129 AA; 14625 MW; 02710F78F1E828AC CRC64; XAGISTFGEV EFTSLETYKQ VAEVNLWGTV RMTKSFLPLI RRAKGEWERE LPPAPEPAPR VHLCSEWKYL SQQLQCPQLS RGRVVNISSM LGRMANPARS PYCITKFGVE AFSDCLRYEM YPLGVKVSV //