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Protein

Hydroxymethylglutaryl-CoA lyase, mitochondrial

Gene

HMGCL

Organism
Bos taurus (Bovine)
Status
Reviewed-Annotation score: -Experimental evidence at transcript leveli

Functioni

Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism (By similarity).By similarity

Catalytic activityi

(S)-3-hydroxy-3-methylglutaryl-CoA = acetyl-CoA + acetoacetate.PROSITE-ProRule annotation

Pathwayi: (S)-3-hydroxy-3-methylglutaryl-CoA degradation

This protein is involved in step 1 of the subpathway that synthesizes acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA.
Proteins known to be involved in this subpathway in this organism are:
  1. Hydroxymethylglutaryl-CoA lyase, mitochondrial (HMGCL)
This subpathway is part of the pathway (S)-3-hydroxy-3-methylglutaryl-CoA degradation, which is itself part of Metabolic intermediate metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA, the pathway (S)-3-hydroxy-3-methylglutaryl-CoA degradation and in Metabolic intermediate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei41SubstrateBy similarity1
Metal bindingi42Divalent metal cationBy similarity1
Metal bindingi233Divalent metal cationBy similarity1
Metal bindingi235Divalent metal cationBy similarity1
Active sitei266PROSITE-ProRule annotation1
Metal bindingi275Divalent metal cationBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLyase
LigandMetal-binding

Enzyme and pathway databases

ReactomeiR-BTA-77111 Synthesis of Ketone Bodies
R-BTA-9033241 Peroxisomal protein import
UniPathwayi
UPA00896;UER00863

Names & Taxonomyi

Protein namesi
Recommended name:
Hydroxymethylglutaryl-CoA lyase, mitochondrial (EC:4.1.3.4)
Short name:
HL
Short name:
HMG-CoA lyase
Alternative name(s):
3-hydroxy-3-methylglutarate-CoA lyase
Gene namesi
Name:HMGCL
OrganismiBos taurus (Bovine)
Taxonomic identifieri9913 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaRuminantiaPecoraBovidaeBovinaeBos
Proteomesi
  • UP000009136 Componenti: Chromosome 2

Organism-specific databases

VGNCiVGNC:29877 HMGCL

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion, Peroxisome

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionBy similarityAdd BLAST27
ChainiPRO_000015151928 – 325Hydroxymethylglutaryl-CoA lyase, mitochondrialAdd BLAST298

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei48N6-acetyllysine; alternateBy similarity1
Modified residuei48N6-succinyllysine; alternateBy similarity1
Modified residuei111N6-acetyllysineBy similarity1
Modified residuei137N6-acetyllysine; alternateBy similarity1
Modified residuei137N6-succinyllysine; alternateBy similarity1
Modified residuei179N6-acetyllysine; alternateBy similarity1
Modified residuei179N6-succinyllysine; alternateBy similarity1
Disulfide bondi323InterchainBy similarity
Modified residuei324N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Disulfide bond

Proteomic databases

PaxDbiQ29448
PeptideAtlasiQ29448
PRIDEiQ29448

Expressioni

Gene expression databases

BgeeiENSBTAG00000021832 Expressed in 10 organ(s), highest expression level in liver

Interactioni

Subunit structurei

Homodimer; disulfide-linked. Can also form homotetramers.By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000029103

Structurei

3D structure databases

ProteinModelPortaliQ29448
SMRiQ29448
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini33 – 300Pyruvate carboxyltransferasePROSITE-ProRule annotationAdd BLAST268

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi323 – 325Microbody targeting signalSequence analysis3

Sequence similaritiesi

Belongs to the HMG-CoA lyase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2368 Eukaryota
COG0119 LUCA
GeneTreeiENSGT00390000017690
HOGENOMiHOG000219757
HOVERGENiHBG064656
InParanoidiQ29448
KOiK01640
OMAiFGGCPMA
OrthoDBiEOG091G0DA9
TreeFamiTF105363

Family and domain databases

Gene3Di3.20.20.70, 1 hit
InterProiView protein in InterPro
IPR013785 Aldolase_TIM
IPR000138 HMG_CoA_lyase_AS
IPR000891 PYR_CT
PfamiView protein in Pfam
PF00682 HMGL-like, 1 hit
PROSITEiView protein in PROSITE
PS01062 HMG_COA_LYASE, 1 hit
PS50991 PYR_CT, 1 hit

Sequencei

Sequence statusi: Complete.

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

Q29448-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MATVKKVLPR RLVGLATLRA VSTSSVGTFP KQVKIVEVGP RDGLQNEKNI
60 70 80 90 100
VPTPVKIKLI DMLSEAGLPV VEATSFVSPK WVPQMADHAE VLKGIQKFPG
110 120 130 140 150
VNYPVLTPNF KGFQAAVAAG AKEVAIFGAA SELFTKKNIN CSIDESLQRF
160 170 180 190 200
DEILKAARAA GISVRGYVSC VLGCPYEGKI SPAKVAEVTK KLYSMGCYEI
210 220 230 240 250
SLGDTIGVGT PGAMKDMLSA VLQEVPVTAL AVHCHDTYGQ ALANTLTALQ
260 270 280 290 300
MGVSVMDSSV AGLGGCPYAQ GASGNLATED LVYMLAGLGI HTGVNLQKLL
310 320
EAGAFICQAL NRRTNSKVAQ ATCKL
Length:325
Mass (Da):34,168
Last modified:October 17, 2006 - v2
Checksum:i4EBB2B79B5547564
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti221V → A in AAA86757 (Ref. 2) Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC118276 mRNA Translation: AAI18277.1
U41409 mRNA Translation: AAA86757.1
RefSeqiNP_001068600.1, NM_001075132.1
UniGeneiBt.9446

Genome annotation databases

EnsembliENSBTAT00000029103; ENSBTAP00000029103; ENSBTAG00000021832
GeneIDi317658
KEGGibta:317658

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC118276 mRNA Translation: AAI18277.1
U41409 mRNA Translation: AAA86757.1
RefSeqiNP_001068600.1, NM_001075132.1
UniGeneiBt.9446

3D structure databases

ProteinModelPortaliQ29448
SMRiQ29448
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000029103

Proteomic databases

PaxDbiQ29448
PeptideAtlasiQ29448
PRIDEiQ29448

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSBTAT00000029103; ENSBTAP00000029103; ENSBTAG00000021832
GeneIDi317658
KEGGibta:317658

Organism-specific databases

CTDi3155
VGNCiVGNC:29877 HMGCL

Phylogenomic databases

eggNOGiKOG2368 Eukaryota
COG0119 LUCA
GeneTreeiENSGT00390000017690
HOGENOMiHOG000219757
HOVERGENiHBG064656
InParanoidiQ29448
KOiK01640
OMAiFGGCPMA
OrthoDBiEOG091G0DA9
TreeFamiTF105363

Enzyme and pathway databases

UniPathwayi
UPA00896;UER00863

ReactomeiR-BTA-77111 Synthesis of Ketone Bodies
R-BTA-9033241 Peroxisomal protein import

Gene expression databases

BgeeiENSBTAG00000021832 Expressed in 10 organ(s), highest expression level in liver

Family and domain databases

Gene3Di3.20.20.70, 1 hit
InterProiView protein in InterPro
IPR013785 Aldolase_TIM
IPR000138 HMG_CoA_lyase_AS
IPR000891 PYR_CT
PfamiView protein in Pfam
PF00682 HMGL-like, 1 hit
PROSITEiView protein in PROSITE
PS01062 HMG_COA_LYASE, 1 hit
PS50991 PYR_CT, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiHMGCL_BOVIN
AccessioniPrimary (citable) accession number: Q29448
Secondary accession number(s): Q148J2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: October 17, 2006
Last modified: September 12, 2018
This is version 120 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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