SDHF4_MOUSE
ID SDHF4_MOUSE Reviewed; 104 AA.
AC Q8BTE0; Q9CQN5; Q9D537;
DT 27-JUN-2006, integrated into UniProtKB/Swiss-Prot.
DT 27-JUN-2006, sequence version 2.
DT 03-AUG-2022, entry version 108.
DE RecName: Full=Succinate dehydrogenase assembly factor 4, mitochondrial {ECO:0000250|UniProtKB:Q5VUM1};
DE Short=SDH assembly factor 4 {ECO:0000250|UniProtKB:Q5VUM1};
DE Short=SDHAF4 {ECO:0000250|UniProtKB:Q5VUM1};
DE Flags: Precursor;
GN Name=Sdhaf4 {ECO:0000250|UniProtKB:Q5VUM1};
OS Mus musculus (Mouse).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae;
OC Murinae; Mus; Mus.
OX NCBI_TaxID=10090;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC STRAIN=C57BL/6J; TISSUE=Pancreas, and Testis;
RX PubMed=16141072; DOI=10.1126/science.1112014;
RA Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N.,
RA Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K.,
RA Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.,
RA Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R.,
RA Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T.,
RA Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A.,
RA Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B.,
RA Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M.,
RA Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S.,
RA Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E.,
RA Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D.,
RA Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M.,
RA Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H.,
RA Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V.,
RA Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S.,
RA Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H.,
RA Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N.,
RA Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F.,
RA Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G.,
RA Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z.,
RA Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C.,
RA Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y.,
RA Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S.,
RA Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K.,
RA Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R.,
RA van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H.,
RA Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M.,
RA Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C.,
RA Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S.,
RA Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K.,
RA Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M.,
RA Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C.,
RA Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A.,
RA Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.;
RT "The transcriptional landscape of the mammalian genome.";
RL Science 309:1559-1563(2005).
RN [2]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC TISSUE=Eye;
RX PubMed=15489334; DOI=10.1101/gr.2596504;
RG The MGC Project Team;
RT "The status, quality, and expansion of the NIH full-length cDNA project:
RT the Mammalian Gene Collection (MGC).";
RL Genome Res. 14:2121-2127(2004).
RN [3]
RP FUNCTION.
RX PubMed=24954416; DOI=10.1016/j.cmet.2014.05.012;
RA Van Vranken J.G., Bricker D.K., Dephoure N., Gygi S.P., Cox J.E.,
RA Thummel C.S., Rutter J.;
RT "SDHAF4 promotes mitochondrial succinate dehydrogenase activity and
RT prevents neurodegeneration.";
RL Cell Metab. 20:241-252(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 (PubMed:24954416). Binds to the flavoprotein
CC subunit Sdha in its FAD-bound form, blocking the generation of excess
CC reactive oxigen species (ROS) and facilitating its assembly with the
CC iron-sulfur protein subunit Sdhb into the SDH catalytic dimer (By
CC similarity). {ECO:0000250|UniProtKB:P38345,
CC ECO:0000269|PubMed:24954416}.
CC -!- SUBUNIT: Interacts with Sdha in its FAD-bound form.
CC {ECO:0000250|UniProtKB:P38345}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion matrix
CC {ECO:0000250|UniProtKB:P38345}.
CC -!- SIMILARITY: Belongs to the SDHAF4 family. {ECO:0000305}.
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DR EMBL; AK003789; BAC25055.1; -; mRNA.
DR EMBL; AK004301; BAB23255.1; -; mRNA.
DR EMBL; AK007909; BAB25342.1; -; mRNA.
DR EMBL; AK015824; BAB29991.1; -; mRNA.
DR EMBL; BC028981; AAH28981.1; -; mRNA.
DR CCDS; CCDS14852.1; -.
DR RefSeq; NP_080779.2; NM_026503.3.
DR AlphaFoldDB; Q8BTE0; -.
DR SMR; Q8BTE0; -.
DR STRING; 10090.ENSMUSP00000027338; -.
DR iPTMnet; Q8BTE0; -.
DR PhosphoSitePlus; Q8BTE0; -.
DR jPOST; Q8BTE0; -.
DR MaxQB; Q8BTE0; -.
DR PaxDb; Q8BTE0; -.
DR PeptideAtlas; Q8BTE0; -.
DR PRIDE; Q8BTE0; -.
DR ProteomicsDB; 256609; -.
DR Antibodypedia; 31244; 42 antibodies from 10 providers.
DR Ensembl; ENSMUST00000027338; ENSMUSP00000027338; ENSMUSG00000026154.
DR GeneID; 68002; -.
DR KEGG; mmu:68002; -.
DR UCSC; uc007amj.2; mouse.
DR CTD; 135154; -.
DR MGI; MGI:1915252; Sdhaf4.
DR VEuPathDB; HostDB:ENSMUSG00000026154; -.
DR eggNOG; KOG3245; Eukaryota.
DR GeneTree; ENSGT00390000009155; -.
DR HOGENOM; CLU_160299_0_0_1; -.
DR InParanoid; Q8BTE0; -.
DR OMA; SLLCHSR; -.
DR OrthoDB; 1530126at2759; -.
DR PhylomeDB; Q8BTE0; -.
DR TreeFam; TF106123; -.
DR BioGRID-ORCS; 68002; 2 hits in 71 CRISPR screens.
DR ChiTaRS; Sdhaf4; mouse.
DR PRO; PR:Q8BTE0; -.
DR Proteomes; UP000000589; Chromosome 1.
DR RNAct; Q8BTE0; protein.
DR Bgee; ENSMUSG00000026154; Expressed in interventricular septum and 251 other tissues.
DR Genevisible; Q8BTE0; MM.
DR GO; GO:0005759; C:mitochondrial matrix; IEA:UniProtKB-SubCell.
DR GO; GO:0005739; C:mitochondrion; HDA:MGI.
DR GO; GO:0008047; F:enzyme activator activity; IMP:UniProtKB.
DR GO; GO:0045333; P:cellular respiration; IMP:UniProtKB.
DR GO; GO:0045087; P:innate immune response; IMP:MGI.
DR GO; GO:0034553; P:mitochondrial respiratory chain complex II assembly; IMP:UniProtKB.
DR GO; GO:1904231; P:positive regulation of succinate dehydrogenase activity; IMP:UniProtKB.
DR InterPro; IPR012875; SDHF4.
DR Pfam; PF07896; DUF1674; 1.
PE 3: Inferred from homology;
KW Chaperone; Mitochondrion; Reference proteome; Transit peptide.
FT TRANSIT 1..30
FT /note="Mitochondrion"
FT /evidence="ECO:0000255"
FT CHAIN 31..104
FT /note="Succinate dehydrogenase assembly factor 4,
FT mitochondrial"
FT /id="PRO_0000244341"
FT REGION 29..104
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 51..104
FT /note="Basic and acidic residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT CONFLICT 17
FT /note="A -> E (in Ref. 1; BAB29991)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 104 AA; 11889 MW; 70ACA3B549C1B916 CRC64;
MVSTTLSVSR MTFVWRAARP SLLNHSLRKM SYQEGKPEPA KQALKKSKLP LGRFDSLEDS
PEEREPLQKF PDDVNPVTKE KGGPKGPEPT RYGDWERKGR CIDF