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P54869

- HMCS2_MOUSE

UniProt

P54869 - HMCS2_MOUSE

Protein

Hydroxymethylglutaryl-CoA synthase, mitochondrial

Gene

Hmgcs2

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 123 (01 Oct 2014)
      Sequence version 2 (15 Mar 2005)
      Previous versions | rss
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    Functioni

    This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.

    Catalytic activityi

    Acetyl-CoA + H2O + acetoacetyl-CoA = (S)-3-hydroxy-3-methylglutaryl-CoA + CoA.PROSITE-ProRule annotation

    Pathwayi

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Binding sitei80 – 801SubstrateBy similarity
    Active sitei132 – 1321Proton donor/acceptorPROSITE-ProRule annotation
    Active sitei166 – 1661Acyl-thioester intermediatePROSITE-ProRule annotation
    Binding sitei204 – 2041SubstrateBy similarity
    Binding sitei258 – 2581SubstrateBy similarity
    Active sitei301 – 3011Proton donor/acceptorPROSITE-ProRule annotation
    Binding sitei380 – 3801SubstrateBy similarity

    GO - Molecular functioni

    1. hydroxymethylglutaryl-CoA synthase activity Source: UniProtKB-EC

    GO - Biological processi

    1. cholesterol biosynthetic process Source: UniProtKB-KW
    2. isoprenoid biosynthetic process Source: InterPro

    Keywords - Molecular functioni

    Transferase

    Keywords - Biological processi

    Cholesterol biosynthesis, Cholesterol metabolism, Lipid biosynthesis, Lipid metabolism, Steroid biosynthesis, Steroid metabolism, Sterol biosynthesis, Sterol metabolism

    Enzyme and pathway databases

    BRENDAi2.3.3.10. 3474.
    ReactomeiREACT_198602. PPARA activates gene expression.
    UniPathwayiUPA00058; UER00102.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Hydroxymethylglutaryl-CoA synthase, mitochondrial (EC:2.3.3.10)
    Short name:
    HMG-CoA synthase
    Alternative name(s):
    3-hydroxy-3-methylglutaryl coenzyme A synthase
    Gene namesi
    Name:Hmgcs2
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 3

    Organism-specific databases

    MGIiMGI:101939. Hmgcs2.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrial inner membrane Source: MGI
    2. mitochondrion Source: MGI

    Keywords - Cellular componenti

    Mitochondrion

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 3737MitochondrionCuratedAdd
    BLAST
    Chaini38 – 508471Hydroxymethylglutaryl-CoA synthase, mitochondrialPRO_0000013484Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei52 – 521N6-succinyllysine1 Publication
    Modified residuei83 – 831N6-acetyllysine; alternate1 Publication
    Modified residuei83 – 831N6-succinyllysine; alternate1 Publication
    Modified residuei118 – 1181N6-acetyllysine; alternate1 Publication
    Modified residuei118 – 1181N6-succinyllysine; alternate1 Publication
    Modified residuei221 – 2211N6-succinyllysine1 Publication
    Modified residuei243 – 2431N6-acetyllysine1 Publication
    Modified residuei256 – 2561N6-acetyllysine; alternate1 Publication
    Modified residuei256 – 2561N6-succinyllysine; alternate1 Publication
    Modified residuei306 – 3061N6-acetyllysine1 Publication
    Modified residuei310 – 3101N6-acetyllysine; alternate1 Publication
    Modified residuei310 – 3101N6-succinyllysine; alternate1 Publication
    Modified residuei327 – 3271N6-acetyllysine; alternate1 Publication
    Modified residuei327 – 3271N6-succinyllysine; alternate1 Publication
    Modified residuei333 – 3331N6-succinyllysine1 Publication
    Modified residuei342 – 3421N6-acetyllysine; alternate1 Publication
    Modified residuei342 – 3421N6-succinyllysine; alternate1 Publication
    Modified residuei350 – 3501N6-acetyllysine; alternate1 Publication
    Modified residuei350 – 3501N6-succinyllysine; alternate1 Publication
    Modified residuei354 – 3541N6-acetyllysine; alternate1 Publication
    Modified residuei354 – 3541N6-succinyllysine; alternate1 Publication
    Modified residuei358 – 3581N6-acetyllysine; alternate1 Publication
    Modified residuei358 – 3581N6-succinyllysine; alternate1 Publication
    Modified residuei427 – 4271N6-acetyllysine1 Publication
    Modified residuei437 – 4371N6-acetyllysine1 Publication
    Modified residuei447 – 4471N6-acetyllysine; alternate1 Publication
    Modified residuei447 – 4471N6-succinyllysine; alternate1 Publication
    Modified residuei473 – 4731N6-acetyllysine; alternate1 Publication
    Modified residuei473 – 4731N6-succinyllysine; alternate1 Publication

    Post-translational modificationi

    Acetylation of Lys-427 is observed in liver mitochondria from fasted mice but not from fed mice.1 Publication

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiP54869.
    PaxDbiP54869.
    PRIDEiP54869.

    2D gel databases

    REPRODUCTION-2DPAGEP54869.
    SWISS-2DPAGEP54869.

    PTM databases

    PhosphoSiteiP54869.

    Expressioni

    Tissue specificityi

    Liver and kidney.

    Gene expression databases

    BgeeiP54869.
    CleanExiMM_HMGCS2.
    GenevestigatoriP54869.

    Interactioni

    Subunit structurei

    Homodimer.By similarity

    Protein-protein interaction databases

    IntActiP54869. 1 interaction.
    MINTiMINT-1859685.

    Structurei

    3D structure databases

    ProteinModelPortaliP54869.
    SMRiP54869. Positions 51-508.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the HMG-CoA synthase family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG3425.
    GeneTreeiENSGT00390000006096.
    HOGENOMiHOG000012351.
    HOVERGENiHBG051912.
    InParanoidiP54869.
    KOiK01641.
    OMAiVDGKYSS.
    OrthoDBiEOG741Z1W.
    PhylomeDBiP54869.
    TreeFamiTF105361.

    Family and domain databases

    Gene3Di3.40.47.10. 1 hit.
    InterProiIPR000590. HMG_CoA_synt_AS.
    IPR013746. HMG_CoA_synt_C_dom.
    IPR013528. HMG_CoA_synth_N.
    IPR010122. HMG_CoA_synthase_euk.
    IPR016039. Thiolase-like.
    IPR016038. Thiolase-like_subgr.
    [Graphical view]
    PfamiPF08540. HMG_CoA_synt_C. 1 hit.
    PF01154. HMG_CoA_synt_N. 1 hit.
    [Graphical view]
    SUPFAMiSSF53901. SSF53901. 3 hits.
    TIGRFAMsiTIGR01833. HMG-CoA-S_euk. 1 hit.
    PROSITEiPS01226. HMG_COA_SYNTHASE. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    P54869-1 [UniParc]FASTAAdd to Basket

    « Hide

    MQRLLAPARR VLQVKRAMQE TSLTPAHLLS AAQQRFSTIP PAPLAKTDTW    50
    PKDVGILALE VYFPAQYVDQ TDLEKFNNVE AGKYTVGLGQ TRMGFCSVQE 100
    DINSLCLTVV QRLMERTKLP WDAVGRLEVG TETIIDKSKA VKTVLMELFQ 150
    DSGNTDIEGI DTTNACYGGT ASLFNAANWM ESSYWDGRYA LVVCGDIAVY 200
    PSGNARPTGG AGAVAMLIGP KAPLVLEQGL RGTHMENAYD FYKPNLASEY 250
    PLVDGKLSIQ CYLRALDRCY AAYRKKIQNQ WKQAGNNQPF TLDDVQYMIF 300
    HTPFCKMVQK SLARLMFNDF LSSSSDKQNN LYKGLEAFRG LKLEETYTNK 350
    DVDKALLKAS LDMFNQKTKA SLYLSTNNGN MYTSSLYGCL ASLLSHHSAQ 400
    ELAGSRIGAF SYGSGLAASF FSFRVSKDAS PGSPLEKLVS SVSDLPKRLD 450
    SRRRMSPEEF TEIMNQREQF YHKVNFSPPG DTSNLFPGTW YLERVDEMHR 500
    RKYARCPV 508
    Length:508
    Mass (Da):56,823
    Last modified:March 15, 2005 - v2
    Checksum:i5A20ACE918FF4758
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti29 – 313LSA → EFR(PubMed:15489334)Curated
    Sequence conflicti394 – 3941L → I in BAB23657. (PubMed:16141072)Curated

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AK004865 mRNA. Translation: BAB23626.1.
    AK004902 mRNA. Translation: BAB23657.1.
    BC014714 mRNA. Translation: AAH14714.1.
    BC024744 mRNA. Translation: AAH24744.1.
    U12790 Genomic DNA. Translation: AAA92675.1.
    U12791 Genomic DNA. Translation: AAA92676.1.
    CCDSiCCDS17662.1.
    PIRiB55729.
    RefSeqiNP_032282.2. NM_008256.4.
    UniGeneiMm.289131.

    Genome annotation databases

    EnsembliENSMUST00000090746; ENSMUSP00000088249; ENSMUSG00000027875.
    ENSMUST00000120541; ENSMUSP00000113296; ENSMUSG00000027875.
    GeneIDi15360.
    KEGGimmu:15360.
    UCSCiuc008qpr.2. mouse.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AK004865 mRNA. Translation: BAB23626.1 .
    AK004902 mRNA. Translation: BAB23657.1 .
    BC014714 mRNA. Translation: AAH14714.1 .
    BC024744 mRNA. Translation: AAH24744.1 .
    U12790 Genomic DNA. Translation: AAA92675.1 .
    U12791 Genomic DNA. Translation: AAA92676.1 .
    CCDSi CCDS17662.1.
    PIRi B55729.
    RefSeqi NP_032282.2. NM_008256.4.
    UniGenei Mm.289131.

    3D structure databases

    ProteinModelPortali P54869.
    SMRi P54869. Positions 51-508.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    IntActi P54869. 1 interaction.
    MINTi MINT-1859685.

    PTM databases

    PhosphoSitei P54869.

    2D gel databases

    REPRODUCTION-2DPAGE P54869.
    SWISS-2DPAGE P54869.

    Proteomic databases

    MaxQBi P54869.
    PaxDbi P54869.
    PRIDEi P54869.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000090746 ; ENSMUSP00000088249 ; ENSMUSG00000027875 .
    ENSMUST00000120541 ; ENSMUSP00000113296 ; ENSMUSG00000027875 .
    GeneIDi 15360.
    KEGGi mmu:15360.
    UCSCi uc008qpr.2. mouse.

    Organism-specific databases

    CTDi 3158.
    MGIi MGI:101939. Hmgcs2.

    Phylogenomic databases

    eggNOGi COG3425.
    GeneTreei ENSGT00390000006096.
    HOGENOMi HOG000012351.
    HOVERGENi HBG051912.
    InParanoidi P54869.
    KOi K01641.
    OMAi VDGKYSS.
    OrthoDBi EOG741Z1W.
    PhylomeDBi P54869.
    TreeFami TF105361.

    Enzyme and pathway databases

    UniPathwayi UPA00058 ; UER00102 .
    BRENDAi 2.3.3.10. 3474.
    Reactomei REACT_198602. PPARA activates gene expression.

    Miscellaneous databases

    ChiTaRSi HMGCS2. mouse.
    NextBioi 287976.
    PROi P54869.
    SOURCEi Search...

    Gene expression databases

    Bgeei P54869.
    CleanExi MM_HMGCS2.
    Genevestigatori P54869.

    Family and domain databases

    Gene3Di 3.40.47.10. 1 hit.
    InterProi IPR000590. HMG_CoA_synt_AS.
    IPR013746. HMG_CoA_synt_C_dom.
    IPR013528. HMG_CoA_synth_N.
    IPR010122. HMG_CoA_synthase_euk.
    IPR016039. Thiolase-like.
    IPR016038. Thiolase-like_subgr.
    [Graphical view ]
    Pfami PF08540. HMG_CoA_synt_C. 1 hit.
    PF01154. HMG_CoA_synt_N. 1 hit.
    [Graphical view ]
    SUPFAMi SSF53901. SSF53901. 3 hits.
    TIGRFAMsi TIGR01833. HMG-CoA-S_euk. 1 hit.
    PROSITEi PS01226. HMG_COA_SYNTHASE. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "The transcriptional landscape of the mammalian genome."
      Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
      , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
      Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Strain: C57BL/6J.
      Tissue: Liver.
    2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Strain: FVB/N.
      Tissue: Colon and Liver.
    3. "Human mitochondrial HMG CoA synthase: liver cDNA and partial genomic cloning, chromosome mapping to 1p12-p13, and possible role in vertebrate evolution."
      Boukaftane Y., Duncan A., Wang S., Labuda D., Robert M.-F., Sarrazin J., Schappert K.T., Mitchell G.A.
      Genomics 23:552-559(1994) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 29-508.
      Strain: CHS.
      Tissue: Liver.
    4. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
      Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
      Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-52; LYS-83; LYS-118; LYS-221; LYS-256; LYS-310; LYS-327; LYS-333; LYS-342; LYS-350; LYS-354; LYS-358; LYS-447 AND LYS-473, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Liver.
    5. "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
      Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
      Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-83; LYS-118; LYS-243; LYS-256; LYS-306; LYS-310; LYS-327; LYS-342; LYS-350; LYS-354; LYS-358; LYS-427; LYS-437; LYS-447 AND LYS-473, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Liver.

    Entry informationi

    Entry nameiHMCS2_MOUSE
    AccessioniPrimary (citable) accession number: P54869
    Secondary accession number(s): Q64740, Q9DBK1, Q9DBM4
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: October 1, 1996
    Last sequence update: March 15, 2005
    Last modified: October 1, 2014
    This is version 123 of the entry and version 2 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. MGD cross-references
      Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
    2. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3