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Protein

Isovaleryl-CoA dehydrogenase, mitochondrial

Gene

Ivd

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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)DescriptionActionsGraphical viewLength
Binding sitei172Substrate; via carbonyl oxygenBy similarity1
Binding sitei275SubstrateBy similarity1
Active sitei284Proton acceptorBy similarity1
Binding sitei310FADBy similarity1
Binding sitei321FADBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi163 – 172FADBy similarity10
Nucleotide bindingi196 – 198FADBy similarity3
Nucleotide bindingi378 – 382FADBy similarity5
Nucleotide bindingi407 – 409FADBy similarity3

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
LigandFAD, 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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 2

Organism-specific databases

MGIiMGI:1929242 Ivd

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

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 30MitochondrionBy similarityAdd BLAST30
ChainiPRO_000000053231 – 424Isovaleryl-CoA dehydrogenase, mitochondrialAdd BLAST394

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei56N6-acetyllysine; alternateCombined sources1
Modified residuei56N6-succinyllysine; alternateCombined sources1
Modified residuei65N6-acetyllysine; alternateCombined sources1
Modified residuei65N6-succinyllysine; alternateCombined sources1
Modified residuei76N6-acetyllysine; alternateCombined sources1
Modified residuei76N6-succinyllysine; alternateCombined sources1
Modified residuei239N6-acetyllysineCombined sources1
Modified residuei260N6-acetyllysine; alternateCombined sources1
Modified residuei260N6-succinyllysine; alternateCombined sources1
Modified residuei316N6-succinyllysineCombined sources1

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
PhosphoSitePlusiQ9JHI5
SwissPalmiQ9JHI5

Expressioni

Gene expression databases

BgeeiENSMUSG00000027332
CleanExiMM_IVD
GenevisibleiQ9JHI5 MM

Interactioni

Subunit structurei

Homotetramer.By similarity

Protein-protein interaction databases

BioGridi207921, 1 interactor
IntActiQ9JHI5, 3 interactors
MINTiQ9JHI5
STRINGi10090.ENSMUSP00000028807

Structurei

3D structure databases

ProteinModelPortaliQ9JHI5
SMRiQ9JHI5
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni220 – 221Substrate bindingBy similarity2
Regioni282 – 285Substrate bindingBy similarity4
Regioni405 – 406Substrate bindingBy similarity2

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
OMAiHNSLCTN
OrthoDBiEOG091G04BS
PhylomeDBiQ9JHI5
TreeFamiTF105050

Family and domain databases

CDDicd01156 IVD, 1 hit
Gene3Di1.10.540.10, 1 hit
InterProiView protein in InterPro
IPR006089 Acyl-CoA_DH_CS
IPR006091 Acyl-CoA_Oxase/DH_cen-dom
IPR036250 AcylCo_DH-like_C
IPR009075 AcylCo_DH/oxidase_C
IPR013786 AcylCoA_DH/ox_N
IPR037069 AcylCoA_DH/ox_N_sf
IPR009100 AcylCoA_DH/oxidase_NM_dom
IPR034183 IVD
PfamiView protein in Pfam
PF00441 Acyl-CoA_dh_1, 1 hit
PF02770 Acyl-CoA_dh_M, 1 hit
PF02771 Acyl-CoA_dh_N, 1 hit
SUPFAMiSSF47203 SSF47203, 1 hit
SSF56645 SSF56645, 1 hit
PROSITEiView protein in PROSITE
PS00072 ACYL_COA_DH_1, 1 hit
PS00073 ACYL_COA_DH_2, 1 hit

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)DescriptionActionsGraphical viewLength
Sequence conflicti29R → G in BAB23751 (PubMed:16141072).Curated1
Sequence conflicti41N → K in BAB30859 (PubMed:16141072).Curated1

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

Similar proteinsi

Entry informationi

Entry nameiIVD_MOUSE
AccessioniPrimary (citable) accession number: Q9JHI5
Secondary accession number(s): Q9CYI3, Q9DBD7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 24, 2005
Last sequence update: October 1, 2000
Last modified: May 23, 2018
This is version 144 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

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