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Protein

Hydroxymethylglutaryl-CoA lyase, mitochondrial

Gene

HMGCL

Organism
Gallus gallus (Chicken)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein 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 sitei14SubstrateBy similarity1
Metal bindingi15Divalent metal cationBy similarity1
Metal bindingi206Divalent metal cationBy similarity1
Metal bindingi208Divalent metal cationBy similarity1
Active sitei239PROSITE-ProRule annotation1
Metal bindingi248Divalent metal cationBy similarity1

GO - Molecular functioni

  • hydroxymethylglutaryl-CoA lyase activity Source: UniProtKB
  • metal ion binding Source: UniProtKB

GO - Biological processi

  • ketone body biosynthetic process Source: UniProtKB

Keywordsi

Molecular functionLyase
LigandMetal-binding

Enzyme and pathway databases

UniPathwayiUPA00896; 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
OrganismiGallus gallus (Chicken)
Taxonomic identifieri9031 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiArchelosauriaArchosauriaDinosauriaSaurischiaTheropodaCoelurosauriaAvesNeognathaeGalloanseraeGalliformesPhasianidaePhasianinaeGallus
Proteomesi
  • UP000000539 Componenti: Unplaced

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
ChainiPRO_00001515201 – 298Hydroxymethylglutaryl-CoA lyase, mitochondrialAdd BLAST298

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei21N6-acetyllysineBy similarity1
Disulfide bondi296InterchainBy similarity

Keywords - PTMi

Acetylation, Disulfide bond

Proteomic databases

PaxDbiP35915.
PRIDEiP35915.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

IntActiP35915. 1 interactor.
STRINGi9031.ENSGALP00000034301.

Structurei

3D structure databases

ProteinModelPortaliP35915.
SMRiP35915.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini6 – 273Pyruvate carboxyltransferasePROSITE-ProRule annotationAdd BLAST268

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi296 – 298Microbody targeting signalSequence analysis3

Sequence similaritiesi

Belongs to the HMG-CoA lyase family.Curated

Phylogenomic databases

eggNOGiKOG2368. Eukaryota.
COG0119. LUCA.
HOGENOMiHOG000219757.
HOVERGENiHBG064656.
InParanoidiP35915.
PhylomeDBiP35915.

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.

P35915-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
AFPQRVKVVE VGPRDGLQNE KSVVPTPVKI RLIDMLSETG LPVIEATSFV
60 70 80 90 100
SPRWVPQMAD HAEVMQGINK LPGVSYPVLT PNLKGFQAAV AAGAKEVSIF
110 120 130 140 150
GAASELFTKK NINCSIEESL ERFSEVMNAA RAASIPVRGY VSCVLGCPYE
160 170 180 190 200
GNISAAKVAE VSKKMYSMGC YEISLGDRIG IGTPGSMKEM LAAVMKEVPV
210 220 230 240 250
GALAVHCHDT YGQALANILV ALQMGVSVVD ASVAGLGGCP YAQGASGNVA
260 270 280 290
TEDLVYMLNG LGIHTGVDLQ KLMDTGTFIC NALNRRTNSK VSQAACRL
Length:298
Mass (Da):31,436
Last modified:June 1, 1994 - v1
Checksum:iA6FCD9418E30590F
GO

Sequence cautioni

The sequence AAA92728 differs from that shown. Reason: Erroneous initiation.Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L07034 mRNA. Translation: AAA92728.1. Different initiation.
PIRiB45470.
UniGeneiGga.2537.

Similar proteinsi

Entry informationi

Entry nameiHMGCL_CHICK
AccessioniPrimary (citable) accession number: P35915
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: June 1, 1994
Last modified: October 25, 2017
This is version 105 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, Direct protein sequencing, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. SIMILARITY comments
    Index of protein domains and families