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Protein

Hydroxymethylglutaryl-CoA lyase, mitochondrial

Gene

HMGCL

Organism
Bos taurus (Bovine)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-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

Complete GO annotation...

Keywords - Molecular functioni

Lyase

Keywords - Ligandi

Metal-binding

Enzyme and pathway databases

ReactomeiR-BTA-77111. Synthesis of Ketone Bodies.
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
OrganismiBos taurus (Bovine)
Taxonomic identifieri9913 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaRuminantiaPecoraBovidaeBovinaeBos
Proteomesi
  • UP000009136 Componenti: Chromosome 2

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

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.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000029103.

Structurei

3D structure databases

ProteinModelPortaliQ29448.
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
Contains 1 pyruvate carboxyltransferase domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2368. Eukaryota.
COG0119. LUCA.
GeneTreeiENSGT00390000017690.
HOGENOMiHOG000219757.
HOVERGENiHBG064656.
InParanoidiQ29448.
KOiK01640.
OMAiEAFAQKN.
OrthoDBiEOG091G0DA9.
TreeFamiTF105363.

Family and domain databases

Gene3Di3.20.20.70. 1 hit.
InterProiIPR013785. Aldolase_TIM.
IPR030020. HL.
IPR000138. HMG_CoA_lyase_AS.
IPR000891. PYR_CT.
[Graphical view]
PANTHERiPTHR10277:SF35. PTHR10277:SF35. 1 hit.
PfamiPF00682. HMGL-like. 1 hit.
[Graphical view]
PROSITEiPS01062. HMG_COA_LYASE. 1 hit.
PS50991. PYR_CT. 1 hit.
[Graphical view]

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.

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.
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.

Phylogenomic databases

eggNOGiKOG2368. Eukaryota.
COG0119. LUCA.
GeneTreeiENSGT00390000017690.
HOGENOMiHOG000219757.
HOVERGENiHBG064656.
InParanoidiQ29448.
KOiK01640.
OMAiEAFAQKN.
OrthoDBiEOG091G0DA9.
TreeFamiTF105363.

Enzyme and pathway databases

UniPathwayiUPA00896; UER00863.
ReactomeiR-BTA-77111. Synthesis of Ketone Bodies.

Gene expression databases

BgeeiENSBTAG00000021832.

Family and domain databases

Gene3Di3.20.20.70. 1 hit.
InterProiIPR013785. Aldolase_TIM.
IPR030020. HL.
IPR000138. HMG_CoA_lyase_AS.
IPR000891. PYR_CT.
[Graphical view]
PANTHERiPTHR10277:SF35. PTHR10277:SF35. 1 hit.
PfamiPF00682. HMGL-like. 1 hit.
[Graphical view]
PROSITEiPS01062. HMG_COA_LYASE. 1 hit.
PS50991. PYR_CT. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiHMGCL_BOVIN
AccessioniPrimary (citable) accession number: Q29448
Secondary accession number(s): Q148J2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: October 17, 2006
Last modified: November 2, 2016
This is version 112 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. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.