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Protein

Isovaleryl-CoA dehydrogenase, mitochondrial

Gene

Ivd

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

Functioni

Catalytic activityi

Isovaleryl-CoA + electron-transfer flavoprotein = 3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein.

Cofactori

Pathwayi: L-leucine degradation

This protein is involved in step 1 of the subpathway that synthesizes (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA.
Proteins known to be involved in the 3 steps of the subpathway in this organism are:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial (Ivd)
  2. Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (Mccc1), Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (Mccc2)
  3. Methylglutaconyl-CoA hydratase, mitochondrial (Auh)
This subpathway is part of the pathway L-leucine degradation, which is itself part of Amino-acid degradation.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA, the pathway L-leucine degradation and in Amino-acid degradation.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei172 – 1721Substrate; via carbonyl oxygenBy similarity
Binding sitei275 – 2751SubstrateBy similarity
Active sitei284 – 2841Proton acceptorBy similarity
Binding sitei310 – 3101FADBy similarity
Binding sitei321 – 3211FADBy similarity

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi163 – 17210FADBy similarity
Nucleotide bindingi196 – 1983FADBy similarity
Nucleotide bindingi378 – 3825FADBy similarity
Nucleotide bindingi407 – 4093FADBy similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

FAD, Flavoprotein

Enzyme and pathway databases

BRENDAi1.3.8.4. 3474.
ReactomeiR-MMU-70895. Branched-chain amino acid catabolism.
UniPathwayiUPA00363; UER00860.

Names & Taxonomyi

Protein namesi
Recommended name:
Isovaleryl-CoA dehydrogenase, mitochondrial (EC:1.3.8.4)
Short name:
IVD
Gene namesi
Name:Ivd
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 2

Organism-specific databases

MGIiMGI:1929242. Ivd.

Subcellular locationi

GO - Cellular componenti

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

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3030MitochondrionBy similarityAdd
BLAST
Chaini31 – 424394Isovaleryl-CoA dehydrogenase, mitochondrialPRO_0000000532Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei56 – 561N6-acetyllysine; alternateCombined sources
Modified residuei56 – 561N6-succinyllysine; alternateCombined sources
Modified residuei65 – 651N6-acetyllysine; alternateCombined sources
Modified residuei65 – 651N6-succinyllysine; alternateCombined sources
Modified residuei76 – 761N6-acetyllysine; alternateCombined sources
Modified residuei76 – 761N6-succinyllysine; alternateCombined sources
Modified residuei239 – 2391N6-acetyllysineCombined sources
Modified residuei260 – 2601N6-acetyllysine; alternateCombined sources
Modified residuei260 – 2601N6-succinyllysine; alternateCombined sources
Modified residuei316 – 3161N6-succinyllysineCombined sources

Post-translational modificationi

Acetylation of Lys-76 is observed in liver mitochondria from fasted mice but not from fed mice.

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ9JHI5.
MaxQBiQ9JHI5.
PaxDbiQ9JHI5.
PeptideAtlasiQ9JHI5.
PRIDEiQ9JHI5.

2D gel databases

REPRODUCTION-2DPAGEIPI00471246.
Q9JHI5.
UCD-2DPAGEQ9JHI5.

PTM databases

iPTMnetiQ9JHI5.
PhosphoSiteiQ9JHI5.
SwissPalmiQ9JHI5.

Expressioni

Gene expression databases

BgeeiENSMUSG00000027332.
CleanExiMM_IVD.
GenevisibleiQ9JHI5. MM.

Interactioni

Subunit structurei

Homotetramer.By similarity

Protein-protein interaction databases

IntActiQ9JHI5. 3 interactions.
MINTiMINT-1861781.
STRINGi10090.ENSMUSP00000028807.

Structurei

3D structure databases

ProteinModelPortaliQ9JHI5.
SMRiQ9JHI5. Positions 36-422.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni220 – 2212Substrate bindingBy similarity
Regioni282 – 2854Substrate bindingBy similarity
Regioni405 – 4062Substrate bindingBy similarity

Sequence similaritiesi

Belongs to the acyl-CoA dehydrogenase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0141. Eukaryota.
ENOG410XNMY. LUCA.
GeneTreeiENSGT00760000119007.
HOGENOMiHOG000131659.
HOVERGENiHBG000224.
InParanoidiQ9JHI5.
KOiK00253.
OMAiMNAARSY.
OrthoDBiEOG091G04BS.
PhylomeDBiQ9JHI5.
TreeFamiTF105050.

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.
PROSITEiPS00072. ACYL_COA_DH_1. 1 hit.
PS00073. ACYL_COA_DH_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

Q9JHI5-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MATAIRLLGR RVSSWRLRPS PSPLAVPRRA HSILPVDDDI NGLNEEQKQL
60 70 80 90 100
RHTISKFLQE NLAPKAQEID QTNDFKNLRE FWKQLGSLGV LGITAPVQYG
110 120 130 140 150
GSGLGYLEHV LVMEEISRAS GAVGLSYGAH SNLCVNQIVR NGNEAQKEKY
160 170 180 190 200
LPKLISGEFI GALAMSEPNA GSDVVSMKLK AEKKGDHYVL NGNKFWITNG
210 220 230 240 250
PDADILVVYA KTDLTAVPAS RGITAFIVEK GMPGFSTSKK LDKLGMRGSN
260 270 280 290 300
TCELVFEDCK VPAANVLSQE SKGVYVLMSG LDLERLVLAG GPLGIMQAVL
310 320 330 340 350
DHTIPYLHVR EAFGQKIGQF QLMQGKMADM YTRLMASRQY VYNVAKACDE
360 370 380 390 400
GHIIPKDCAG VILYAAECAT QVALDGIQCL GGNGYINDFP MGRFLRDAKL
410 420
YEIGAGTSEV RRLVIGRAFN ADFR
Length:424
Mass (Da):46,325
Last modified:October 1, 2000 - v1
Checksum:i7B8A556A9E73B0B6
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti29 – 291R → G in BAB23751 (PubMed:16141072).Curated
Sequence conflicti41 – 411N → K in BAB30859 (PubMed:16141072).Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF225989 mRNA. Translation: AAF35888.1.
AF226043
, AF226039, AF226041, AF226040, AF226042 Genomic DNA. Translation: AAF67667.1.
AK005024 mRNA. Translation: BAB23751.1.
AK017660 mRNA. Translation: BAB30859.1.
BC018325 mRNA. Translation: AAH18325.1.
BC027198 mRNA. Translation: AAH27198.1.
CCDSiCCDS16586.1.
RefSeqiNP_062800.1. NM_019826.3.
UniGeneiMm.6635.

Genome annotation databases

EnsembliENSMUST00000028807; ENSMUSP00000028807; ENSMUSG00000027332.
GeneIDi56357.
KEGGimmu:56357.
UCSCiuc008lsv.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF225989 mRNA. Translation: AAF35888.1.
AF226043
, AF226039, AF226041, AF226040, AF226042 Genomic DNA. Translation: AAF67667.1.
AK005024 mRNA. Translation: BAB23751.1.
AK017660 mRNA. Translation: BAB30859.1.
BC018325 mRNA. Translation: AAH18325.1.
BC027198 mRNA. Translation: AAH27198.1.
CCDSiCCDS16586.1.
RefSeqiNP_062800.1. NM_019826.3.
UniGeneiMm.6635.

3D structure databases

ProteinModelPortaliQ9JHI5.
SMRiQ9JHI5. Positions 36-422.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ9JHI5. 3 interactions.
MINTiMINT-1861781.
STRINGi10090.ENSMUSP00000028807.

PTM databases

iPTMnetiQ9JHI5.
PhosphoSiteiQ9JHI5.
SwissPalmiQ9JHI5.

2D gel databases

REPRODUCTION-2DPAGEIPI00471246.
Q9JHI5.
UCD-2DPAGEQ9JHI5.

Proteomic databases

EPDiQ9JHI5.
MaxQBiQ9JHI5.
PaxDbiQ9JHI5.
PeptideAtlasiQ9JHI5.
PRIDEiQ9JHI5.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000028807; ENSMUSP00000028807; ENSMUSG00000027332.
GeneIDi56357.
KEGGimmu:56357.
UCSCiuc008lsv.1. mouse.

Organism-specific databases

CTDi3712.
MGIiMGI:1929242. Ivd.

Phylogenomic databases

eggNOGiKOG0141. Eukaryota.
ENOG410XNMY. LUCA.
GeneTreeiENSGT00760000119007.
HOGENOMiHOG000131659.
HOVERGENiHBG000224.
InParanoidiQ9JHI5.
KOiK00253.
OMAiMNAARSY.
OrthoDBiEOG091G04BS.
PhylomeDBiQ9JHI5.
TreeFamiTF105050.

Enzyme and pathway databases

UniPathwayiUPA00363; UER00860.
BRENDAi1.3.8.4. 3474.
ReactomeiR-MMU-70895. Branched-chain amino acid catabolism.

Miscellaneous databases

PROiQ9JHI5.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000027332.
CleanExiMM_IVD.
GenevisibleiQ9JHI5. 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.
PROSITEiPS00072. ACYL_COA_DH_1. 1 hit.
PS00073. ACYL_COA_DH_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiIVD_MOUSE
AccessioniPrimary (citable) accession number: Q9JHI5
Secondary accession number(s): Q9CYI3, Q9DBD7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 24, 2005
Last sequence update: October 1, 2000
Last modified: September 7, 2016
This is version 133 of the entry and version 1 of the sequence. [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. 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.