SDHF1_YEAST
ID SDHF1_YEAST Reviewed; 79 AA.
AC Q3E785; D6VT12;
DT 17-OCT-2006, integrated into UniProtKB/Swiss-Prot.
DT 08-NOV-2005, sequence version 1.
DT 03-AUG-2022, entry version 109.
DE RecName: Full=Succinate dehydrogenase assembly factor 1, mitochondrial {ECO:0000303|PubMed:19465911};
DE Short=SDH assembly factor 1 {ECO:0000303|PubMed:19465911};
DE Short=SDHAF1 {ECO:0000303|PubMed:19465911};
GN Name=SDH6 {ECO:0000303|PubMed:24954417};
GN OrderedLocusNames=YDR379C-A {ECO:0000312|SGD:S000007605};
GN ORFNames=smORF139 {ECO:0000303|PubMed:12566404},
GN smORF8 {ECO:0000303|PubMed:12566404};
OS Saccharomyces cerevisiae (strain ATCC 204508 / S288c) (Baker's yeast).
OC Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes;
OC Saccharomycetales; Saccharomycetaceae; Saccharomyces.
OX NCBI_TaxID=559292;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RC STRAIN=ATCC 204508 / S288c;
RX PubMed=9169867;
RA Jacq C., Alt-Moerbe J., Andre B., Arnold W., Bahr A., Ballesta J.P.G.,
RA Bargues M., Baron L., Becker A., Biteau N., Bloecker H., Blugeon C.,
RA Boskovic J., Brandt P., Brueckner M., Buitrago M.J., Coster F.,
RA Delaveau T., del Rey F., Dujon B., Eide L.G., Garcia-Cantalejo J.M.,
RA Goffeau A., Gomez-Peris A., Granotier C., Hanemann V., Hankeln T.,
RA Hoheisel J.D., Jaeger W., Jimenez A., Jonniaux J.-L., Kraemer C.,
RA Kuester H., Laamanen P., Legros Y., Louis E.J., Moeller-Rieker S.,
RA Monnet A., Moro M., Mueller-Auer S., Nussbaumer B., Paricio N., Paulin L.,
RA Perea J., Perez-Alonso M., Perez-Ortin J.E., Pohl T.M., Prydz H.,
RA Purnelle B., Rasmussen S.W., Remacha M.A., Revuelta J.L., Rieger M.,
RA Salom D., Saluz H.P., Saiz J.E., Saren A.-M., Schaefer M., Scharfe M.,
RA Schmidt E.R., Schneider C., Scholler P., Schwarz S., Soler-Mira A.,
RA Urrestarazu L.A., Verhasselt P., Vissers S., Voet M., Volckaert G.,
RA Wagner G., Wambutt R., Wedler E., Wedler H., Woelfl S., Harris D.E.,
RA Bowman S., Brown D., Churcher C.M., Connor R., Dedman K., Gentles S.,
RA Hamlin N., Hunt S., Jones L., McDonald S., Murphy L.D., Niblett D.,
RA Odell C., Oliver K., Rajandream M.A., Richards C., Shore L., Walsh S.V.,
RA Barrell B.G., Dietrich F.S., Mulligan J.T., Allen E., Araujo R., Aviles E.,
RA Berno A., Carpenter J., Chen E., Cherry J.M., Chung E., Duncan M.,
RA Hunicke-Smith S., Hyman R.W., Komp C., Lashkari D., Lew H., Lin D.,
RA Mosedale D., Nakahara K., Namath A., Oefner P., Oh C., Petel F.X.,
RA Roberts D., Schramm S., Schroeder M., Shogren T., Shroff N., Winant A.,
RA Yelton M.A., Botstein D., Davis R.W., Johnston M., Andrews S., Brinkman R.,
RA Cooper J., Ding H., Du Z., Favello A., Fulton L., Gattung S., Greco T.,
RA Hallsworth K., Hawkins J., Hillier L.W., Jier M., Johnson D., Johnston L.,
RA Kirsten J., Kucaba T., Langston Y., Latreille P., Le T., Mardis E.,
RA Menezes S., Miller N., Nhan M., Pauley A., Peluso D., Rifkin L., Riles L.,
RA Taich A., Trevaskis E., Vignati D., Wilcox L., Wohldman P., Vaudin M.,
RA Wilson R., Waterston R., Albermann K., Hani J., Heumann K., Kleine K.,
RA Mewes H.-W., Zollner A., Zaccaria P.;
RT "The nucleotide sequence of Saccharomyces cerevisiae chromosome IV.";
RL Nature 387:75-78(1997).
RN [2]
RP GENOME REANNOTATION.
RC STRAIN=ATCC 204508 / S288c;
RX PubMed=24374639; DOI=10.1534/g3.113.008995;
RA Engel S.R., Dietrich F.S., Fisk D.G., Binkley G., Balakrishnan R.,
RA Costanzo M.C., Dwight S.S., Hitz B.C., Karra K., Nash R.S., Weng S.,
RA Wong E.D., Lloyd P., Skrzypek M.S., Miyasato S.R., Simison M., Cherry J.M.;
RT "The reference genome sequence of Saccharomyces cerevisiae: Then and now.";
RL G3 (Bethesda) 4:389-398(2014).
RN [3]
RP GENOME REANNOTATION.
RX PubMed=11152879; DOI=10.1016/s0014-5793(00)02275-4;
RA Blandin G., Durrens P., Tekaia F., Aigle M., Bolotin-Fukuhara M., Bon E.,
RA Casaregola S., de Montigny J., Gaillardin C., Lepingle A., Llorente B.,
RA Malpertuy A., Neuveglise C., Ozier-Kalogeropoulos O., Perrin A., Potier S.,
RA Souciet J.-L., Talla E., Toffano-Nioche C., Wesolowski-Louvel M., Marck C.,
RA Dujon B.;
RT "Genomic exploration of the hemiascomycetous yeasts: 4. The genome of
RT Saccharomyces cerevisiae revisited.";
RL FEBS Lett. 487:31-36(2000).
RN [4]
RP GENOME REANNOTATION.
RX PubMed=12566404; DOI=10.1101/gr.232903;
RA Kessler M.M., Zeng Q., Hogan S., Cook R., Morales A.J., Cottarel G.;
RT "Systematic discovery of new genes in the Saccharomyces cerevisiae
RT genome.";
RL Genome Res. 13:264-271(2003).
RN [5]
RP SUBCELLULAR LOCATION [LARGE SCALE ANALYSIS].
RX PubMed=14562095; DOI=10.1038/nature02026;
RA Huh W.-K., Falvo J.V., Gerke L.C., Carroll A.S., Howson R.W.,
RA Weissman J.S., O'Shea E.K.;
RT "Global analysis of protein localization in budding yeast.";
RL Nature 425:686-691(2003).
RN [6]
RP LEVEL OF PROTEIN EXPRESSION [LARGE SCALE ANALYSIS].
RX PubMed=14562106; DOI=10.1038/nature02046;
RA Ghaemmaghami S., Huh W.-K., Bower K., Howson R.W., Belle A., Dephoure N.,
RA O'Shea E.K., Weissman J.S.;
RT "Global analysis of protein expression in yeast.";
RL Nature 425:737-741(2003).
RN [7]
RP FUNCTION, DISRUPTION PHENOTYPE, AND MUTAGENESIS OF ARG-61 AND GLY-63.
RX PubMed=19465911; DOI=10.1038/ng.378;
RA Ghezzi D., Goffrini P., Uziel G., Horvath R., Klopstock T., Lochmuller H.,
RA D'Adamo P., Gasparini P., Strom T.M., Prokisch H., Invernizzi F.,
RA Ferrero I., Zeviani M.;
RT "SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in
RT SDH-defective infantile leukoencephalopathy.";
RL Nat. Genet. 41:654-656(2009).
RN [8]
RP FUNCTION, AND INTERACTION WITH SDH2.
RX PubMed=24954417; DOI=10.1016/j.cmet.2014.05.014;
RA Na U., Yu W., Cox J., Bricker D.K., Brockmann K., Rutter J., Thummel C.S.,
RA Winge D.R.;
RT "The LYR factors SDHAF1 and SDHAF3 mediate maturation of the iron-sulfur
RT subunit of succinate dehydrogenase.";
RL Cell Metab. 20:253-266(2014).
CC -!- FUNCTION: Plays an essential role in the assembly of succinate
CC dehydrogenase (SDH), an enzyme complex (also referred to as respiratory
CC complex II) that is a component of both the tricarboxylic acid (TCA)
CC cycle and the mitochondrial electron transport chain, and which couples
CC the oxidation of succinate to fumarate with the reduction of ubiquinone
CC (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur
CC protein subunit SDH2 of the SDH catalytic dimer, protecting it from the
CC deleterious effects of oxidants. Acts together with SDHAF3 (SDH7).
CC {ECO:0000269|PubMed:19465911}.
CC -!- SUBUNIT: Interacts with SDH2 within an SDH1-SDH2 subcomplex.
CC {ECO:0000269|PubMed:24954417}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion matrix
CC {ECO:0000269|PubMed:14562095}.
CC -!- DISRUPTION PHENOTYPE: OXPHOS incompetent because of a profound and
CC specific reduction of complex II activity.
CC {ECO:0000269|PubMed:19465911}.
CC -!- MISCELLANEOUS: Present with 861 molecules/cell in log phase SD medium.
CC {ECO:0000269|PubMed:14562106}.
CC -!- SIMILARITY: Belongs to the complex I LYR family. SDHAF1 subfamily.
CC {ECO:0000305}.
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DR EMBL; U28373; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR EMBL; BK006938; DAA12222.1; -; Genomic_DNA.
DR RefSeq; NP_076888.3; NM_001184471.3.
DR AlphaFoldDB; Q3E785; -.
DR SMR; Q3E785; -.
DR BioGRID; 32439; 44.
DR STRING; 4932.YDR379C-A; -.
DR MaxQB; Q3E785; -.
DR PaxDb; Q3E785; -.
DR PRIDE; Q3E785; -.
DR EnsemblFungi; YDR379C-A_mRNA; YDR379C-A; YDR379C-A.
DR GeneID; 851986; -.
DR KEGG; sce:YDR379C-A; -.
DR SGD; S000007605; SDH6.
DR VEuPathDB; FungiDB:YDR379C-A; -.
DR eggNOG; KOG4620; Eukaryota.
DR HOGENOM; CLU_154777_1_1_1; -.
DR InParanoid; Q3E785; -.
DR OMA; GQRRYET; -.
DR BioCyc; YEAST:G3O-30111-MON; -.
DR PRO; PR:Q3E785; -.
DR Proteomes; UP000002311; Chromosome IV.
DR RNAct; Q3E785; protein.
DR GO; GO:0005759; C:mitochondrial matrix; IEA:UniProtKB-SubCell.
DR GO; GO:0005739; C:mitochondrion; HDA:SGD.
DR GO; GO:0016226; P:iron-sulfur cluster assembly; NAS:UniProtKB.
DR GO; GO:0034553; P:mitochondrial respiratory chain complex II assembly; IMP:UniProtKB.
DR CDD; cd20268; Complex1_LYR_SDHAF1_LYRM8; 1.
DR InterPro; IPR008011; Complex1_LYR_dom.
DR InterPro; IPR045295; Complex1_LYR_SDHAF1_LYRM8.
DR Pfam; PF05347; Complex1_LYR; 1.
PE 1: Evidence at protein level;
KW Chaperone; Mitochondrion; Reference proteome.
FT CHAIN 1..79
FT /note="Succinate dehydrogenase assembly factor 1,
FT mitochondrial"
FT /id="PRO_0000253849"
FT MUTAGEN 61
FT /note="R->P: Abolishes the ability to complement a yeast
FT strain carrying a deletion for this gene."
FT /evidence="ECO:0000269|PubMed:19465911"
FT MUTAGEN 63
FT /note="G->R: Abolishes the ability to complement a yeast
FT strain carrying a deletion for this gene."
FT /evidence="ECO:0000269|PubMed:19465911"
SQ SEQUENCE 79 AA; 9352 MW; 2A49008969061739 CRC64;
MPKRLSGLQK EVLHLYRASI RTAHTKPKEN QVNFVNYIHE EFGKYRNLPR KDFTTIEHLL
RVGNKKIATF SHPELTNIH
