Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Glutaryl-CoA dehydrogenase, mitochondrial

Gene

Gcdh

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO2 in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor.

Catalytic activityi

Glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein.

Cofactori

Pathwayi: lysine degradation

This protein is involved in the pathway lysine degradation, which is part of Amino-acid metabolism.
View all proteins of this organism that are known to be involved in the pathway lysine degradation and in Amino-acid metabolism.

Pathwayi: tryptophan metabolism

This protein is involved in the pathway tryptophan metabolism, which is part of Amino-acid metabolism.
View all proteins of this organism that are known to be involved in the pathway tryptophan metabolism and in Amino-acid metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei186FADBy similarity1
Binding sitei186Substrate; via carbonyl oxygenBy similarity1
Binding sitei294SubstrateBy similarity1
Binding sitei319FADBy similarity1
Binding sitei330FADBy similarity1
Active sitei414Proton acceptorBy similarity1
Binding sitei415Substrate; via amide nitrogenBy similarity1
Binding sitei416FADBy similarity1
Binding sitei434FAD; via carbonyl oxygenBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi177 – 186FADBy similarity10
Nucleotide bindingi177 – 180FADBy similarity4
Nucleotide bindingi212 – 214FADBy similarity3
Nucleotide bindingi387 – 391FADBy similarity5
Nucleotide bindingi416 – 418FADBy similarity3

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

FAD, Flavoprotein

Enzyme and pathway databases

BRENDAi1.3.8.6. 3474.
ReactomeiR-MMU-71064. Lysine catabolism.
UniPathwayiUPA00224.
UPA00225.

Names & Taxonomyi

Protein namesi
Recommended name:
Glutaryl-CoA dehydrogenase, mitochondrial (EC:1.3.8.6)
Short name:
GCD
Gene namesi
Name:Gcdh
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 8

Organism-specific databases

MGIiMGI:104541. Gcdh.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial inner membrane Source: MGI
  • mitochondrial matrix Source: UniProtKB-SubCell
  • mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Disruption phenotypei

Animals show highly increased protein glutarylation in liver.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 44MitochondrionSequence analysisAdd BLAST44
ChainiPRO_000000052845 – 438Glutaryl-CoA dehydrogenase, mitochondrialAdd BLAST394

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei51N6-acetyllysine; alternateCombined sources1
Modified residuei51N6-succinyllysine; alternateCombined sources1
Modified residuei65N6-acetyllysineCombined sources1
Modified residuei240N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ60759.
MaxQBiQ60759.
PaxDbiQ60759.
PeptideAtlasiQ60759.
PRIDEiQ60759.

PTM databases

iPTMnetiQ60759.
PhosphoSitePlusiQ60759.

Expressioni

Gene expression databases

BgeeiENSMUSG00000003809.
CleanExiMM_GCDH.
ExpressionAtlasiQ60759. baseline and differential.
GenevisibleiQ60759. MM.

Interactioni

Subunit structurei

Homotetramer.

Protein-protein interaction databases

BioGridi234755. 1 interactor.
IntActiQ60759. 2 interactors.
MINTiMINT-1861107.
STRINGi10090.ENSMUSP00000003907.

Structurei

3D structure databases

ProteinModelPortaliQ60759.
SMRiQ60759.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni138 – 139Substrate bindingBy similarity2
Regioni287 – 294Substrate bindingBy similarity8

Sequence similaritiesi

Belongs to the acyl-CoA dehydrogenase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0138. Eukaryota.
COG1960. LUCA.
GeneTreeiENSGT00760000119007.
HOGENOMiHOG000131662.
HOVERGENiHBG001939.
InParanoidiQ60759.
KOiK00252.
TreeFamiTF105051.

Family and domain databases

Gene3Di1.10.540.10. 1 hit.
InterProiIPR006089. Acyl-CoA_DH_CS.
IPR006091. Acyl-CoA_Oxase/DH_cen-dom.
IPR009075. AcylCo_DH/oxidase_C.
IPR013786. AcylCoA_DH/ox_N.
IPR009100. AcylCoA_DH/oxidase_NM_dom.
[Graphical view]
PfamiPF00441. Acyl-CoA_dh_1. 1 hit.
PF02770. Acyl-CoA_dh_M. 1 hit.
PF02771. Acyl-CoA_dh_N. 1 hit.
[Graphical view]
SUPFAMiSSF47203. SSF47203. 1 hit.
SSF56645. SSF56645. 1 hit.
PROSITEiPS00073. ACYL_COA_DH_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

Q60759-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSLRGVSARL LSRRSGLRFP RFPRTWSSAA AHTEKTQIRP AKSSRPVFDW
60 70 80 90 100
KDPLILEEQL TADEKLIRDT FRNYCQERLM SRILLANRNE VFHRDIVYEM
110 120 130 140 150
GELGVLGPTI KGYGCAGVSS VAYGLLTREL ERVDSGYRSM MSVQSSLVMH
160 170 180 190 200
PIYTYGSEEQ RQKYLPGLAK GELLGCFGLT EPNHGSDPGG METRARHNPS
210 220 230 240 250
NQSYTLSGTK TWITNSPVAD LFIVWARCED NCIRGFILEK GMRGLSAPRI
260 270 280 290 300
EGKFSLRASA TGMIIMDSVE VPEENVLPNV SSLAGPFGCL NTARYGITWG
310 320 330 340 350
VLGAAEFCLH TARQYALDRI QFGVPLARNQ LVQKKLADML TEITLGLHAC
360 370 380 390 400
LQLGRLKDQD KATPEMVSML KRNNCGKALD IARQARDILG GNGISDEYHV
410 420 430
IRHAMNLEAV NTYEGTHDIH ALILGRAITG IQAFTVGK
Length:438
Mass (Da):48,606
Last modified:July 27, 2011 - v2
Checksum:iA904457D76F2BD90
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti9R → Q in AAB04679 (PubMed:7490088).Curated1
Sequence conflicti75C → W in AAB04679 (PubMed:7490088).Curated1
Sequence conflicti82R → Q in AAB04679 (PubMed:7490088).Curated1
Sequence conflicti167G → R in AAB04679 (PubMed:7490088).Curated1
Sequence conflicti234R → P in AAB04679 (PubMed:7490088).Curated1
Sequence conflicti245L → S in AAB04679 (PubMed:7490088).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U18992 mRNA. Translation: AAB04679.1.
AK165406 mRNA. Translation: BAE38166.1.
BC061158 mRNA. Translation: AAH61158.1.
RefSeqiNP_032123.3. NM_008097.2.
UniGeneiMm.2475.

Genome annotation databases

EnsembliENSMUST00000109745; ENSMUSP00000105367; ENSMUSG00000003809.
GeneIDi270076.
KEGGimmu:270076.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U18992 mRNA. Translation: AAB04679.1.
AK165406 mRNA. Translation: BAE38166.1.
BC061158 mRNA. Translation: AAH61158.1.
RefSeqiNP_032123.3. NM_008097.2.
UniGeneiMm.2475.

3D structure databases

ProteinModelPortaliQ60759.
SMRiQ60759.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi234755. 1 interactor.
IntActiQ60759. 2 interactors.
MINTiMINT-1861107.
STRINGi10090.ENSMUSP00000003907.

PTM databases

iPTMnetiQ60759.
PhosphoSitePlusiQ60759.

Proteomic databases

EPDiQ60759.
MaxQBiQ60759.
PaxDbiQ60759.
PeptideAtlasiQ60759.
PRIDEiQ60759.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000109745; ENSMUSP00000105367; ENSMUSG00000003809.
GeneIDi270076.
KEGGimmu:270076.

Organism-specific databases

CTDi2639.
MGIiMGI:104541. Gcdh.

Phylogenomic databases

eggNOGiKOG0138. Eukaryota.
COG1960. LUCA.
GeneTreeiENSGT00760000119007.
HOGENOMiHOG000131662.
HOVERGENiHBG001939.
InParanoidiQ60759.
KOiK00252.
TreeFamiTF105051.

Enzyme and pathway databases

UniPathwayiUPA00224.
UPA00225.
BRENDAi1.3.8.6. 3474.
ReactomeiR-MMU-71064. Lysine catabolism.

Miscellaneous databases

PROiQ60759.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000003809.
CleanExiMM_GCDH.
ExpressionAtlasiQ60759. baseline and differential.
GenevisibleiQ60759. MM.

Family and domain databases

Gene3Di1.10.540.10. 1 hit.
InterProiIPR006089. Acyl-CoA_DH_CS.
IPR006091. Acyl-CoA_Oxase/DH_cen-dom.
IPR009075. AcylCo_DH/oxidase_C.
IPR013786. AcylCoA_DH/ox_N.
IPR009100. AcylCoA_DH/oxidase_NM_dom.
[Graphical view]
PfamiPF00441. Acyl-CoA_dh_1. 1 hit.
PF02770. Acyl-CoA_dh_M. 1 hit.
PF02771. Acyl-CoA_dh_N. 1 hit.
[Graphical view]
SUPFAMiSSF47203. SSF47203. 1 hit.
SSF56645. SSF56645. 1 hit.
PROSITEiPS00073. ACYL_COA_DH_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiGCDH_MOUSE
AccessioniPrimary (citable) accession number: Q60759
Secondary accession number(s): Q6P8N6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: July 27, 2011
Last modified: November 2, 2016
This is version 135 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

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.