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Protein

Hydroxymethylglutaryl-CoA lyase, mitochondrial

Gene

Hmgcl

Organism
Rattus norvegicus (Rat)
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 similarity1 Publication

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. 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic (Hmgcll1), 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

BRENDAi4.1.3.4 5301
ReactomeiR-RNO-77111 Synthesis of Ketone Bodies
R-RNO-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
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 5

Organism-specific databases

RGDi620554 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_000001348228 – 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

PaxDbiP97519
PRIDEiP97519

PTM databases

iPTMnetiP97519
PhosphoSitePlusiP97519

Expressioni

Tissue specificityi

In suckling rat, highest levels in liver and in intestine. Lower levels in heart, kidney and cerebellum. Weak expression in brain cortex, medulla and midbrain. Levels decrease slightly during weaning.1 Publication

Gene expression databases

BgeeiENSRNOG00000009422 Expressed in 10 organ(s), highest expression level in liver
GenevisibleiP97519 RN

Interactioni

Subunit structurei

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

GO - Molecular functioni

Protein-protein interaction databases

IntActiP97519, 1 interactor
STRINGi10116.ENSRNOP00000012853

Structurei

3D structure databases

ProteinModelPortaliP97519
SMRiP97519
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
InParanoidiP97519
KOiK01640
OMAiFGGCPMA
OrthoDBiEOG091G0DA9
PhylomeDBiP97519
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.

P97519-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MATVRKAFPQ RLVGLASLRA ASTSSMGTLP KRVKIVEVGP RDGLQNEKSI
60 70 80 90 100
VPTPVKIKLI DMLSEAGLPV IEATSFVSPK WVPQMADHSD VLKGIQKFPG
110 120 130 140 150
INYPVLTPNM KGFEEAVAAG AKEVSIFGAA SELFTRKNVN CSIEESFQRF
160 170 180 190 200
DGVMQAARAA SISVRGYVSC ALGCPYEGKV SPAKVAEVAK KLYSMGCYEI
210 220 230 240 250
SLGDTIGVGT PGLMKDMLTA VLHEVPVAAL AVHCHDTYGQ ALANTLVALQ
260 270 280 290 300
MGVSVVDSSV AGLGGCPYAK GASGNLATED LVYMLTGLGI HTGVNLQKLL
310 320
EAGDFICQAL NRKTSSKVAQ ATCKL
Length:325
Mass (Da):34,192
Last modified:May 1, 1997 - v1
Checksum:i3557684CF457D19E
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y10054 mRNA Translation: CAA71148.1
BC061797 mRNA Translation: AAH61797.1
RefSeqiNP_077362.1, NM_024386.1
UniGeneiRn.12297

Genome annotation databases

EnsembliENSRNOT00000012853; ENSRNOP00000012853; ENSRNOG00000009422
GeneIDi79238
KEGGirno:79238
UCSCiRGD:620554 rat

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y10054 mRNA Translation: CAA71148.1
BC061797 mRNA Translation: AAH61797.1
RefSeqiNP_077362.1, NM_024386.1
UniGeneiRn.12297

3D structure databases

ProteinModelPortaliP97519
SMRiP97519
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP97519, 1 interactor
STRINGi10116.ENSRNOP00000012853

PTM databases

iPTMnetiP97519
PhosphoSitePlusiP97519

Proteomic databases

PaxDbiP97519
PRIDEiP97519

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSRNOT00000012853; ENSRNOP00000012853; ENSRNOG00000009422
GeneIDi79238
KEGGirno:79238
UCSCiRGD:620554 rat

Organism-specific databases

CTDi3155
RGDi620554 Hmgcl

Phylogenomic databases

eggNOGiKOG2368 Eukaryota
COG0119 LUCA
GeneTreeiENSGT00390000017690
HOGENOMiHOG000219757
HOVERGENiHBG064656
InParanoidiP97519
KOiK01640
OMAiFGGCPMA
OrthoDBiEOG091G0DA9
PhylomeDBiP97519
TreeFamiTF105363

Enzyme and pathway databases

UniPathwayi
UPA00896;UER00863

BRENDAi4.1.3.4 5301
ReactomeiR-RNO-77111 Synthesis of Ketone Bodies
R-RNO-9033241 Peroxisomal protein import

Miscellaneous databases

PROiPR:P97519

Gene expression databases

BgeeiENSRNOG00000009422 Expressed in 10 organ(s), highest expression level in liver
GenevisibleiP97519 RN

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_RAT
AccessioniPrimary (citable) accession number: P97519
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: May 1, 1997
Last modified: September 12, 2018
This is version 129 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
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Main funding by: National Institutes of Health

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