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P54869

- HMCS2_MOUSE

UniProt

P54869 - HMCS2_MOUSE

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Protein
Hydroxymethylglutaryl-CoA synthase, mitochondrial
Gene
Hmgcs2
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

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.

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei80 – 801Substrate By similarity
Active sitei132 – 1321Proton donor/acceptor By similarity
Active sitei166 – 1661Acyl-thioester intermediate By similarity
Binding sitei204 – 2041Substrate By similarity
Binding sitei258 – 2581Substrate By similarity
Active sitei301 – 3011Proton donor/acceptor By similarity
Binding sitei380 – 3801Substrate By similarity

GO - Molecular functioni

  1. hydroxymethylglutaryl-CoA synthase activity Source: UniProtKB-EC
Complete GO annotation...

GO - Biological processi

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

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
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3737Mitochondrion Inferred
Add
BLAST
Chaini38 – 508471Hydroxymethylglutaryl-CoA synthase, mitochondrial
PRO_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.

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.

Family & Domainsi

Sequence similaritiesi

Belongs to the HMG-CoA synthase family.

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

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti29 – 313LSA → EFR1 Publication
Sequence conflicti394 – 3941L → I in BAB23657. 1 Publication

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...

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

« Hide 'large scale' publications
  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: September 3, 2014
This is version 122 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

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