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Protein

Dimethylglycine dehydrogenase, mitochondrial

Gene

Dmgdh

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the demethylation of N,N-dimethylglycine to sarcosine. Also has activity with sarcosine in vitro.By similarity

Catalytic activityi

N,N-dimethylglycine + electron-transfer flavoprotein + H2O = sarcosine + formaldehyde + reduced electron-transfer flavoprotein.By similarity

Cofactori

FADBy similarityNote: Binds 1 FAD covalently per monomer.By similarity

Pathwayi: betaine degradation

This protein is involved in step 2 of the subpathway that synthesizes sarcosine from betaine.
Proteins known to be involved in the 2 steps of the subpathway in this organism are:
  1. Betaine--homocysteine S-methyltransferase 1 (Bhmt)
  2. Dimethylglycine dehydrogenase, mitochondrial (Dmgdh)
This subpathway is part of the pathway betaine degradation, which is itself part of Amine and polyamine degradation.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes sarcosine from betaine, the pathway betaine degradation and in Amine and polyamine degradation.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei212FAD; via amide nitrogen and carbonyl oxygenBy similarity1
Binding sitei244FADBy similarity1
Binding sitei669TetrahydrofolateBy similarity1
Binding sitei737TetrahydrofolateBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi52 – 53FADBy similarity2
Nucleotide bindingi73 – 74FADBy similarity2
Nucleotide bindingi80 – 88FADBy similarity9
Nucleotide bindingi390 – 395FADBy similarity6

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
LigandFAD, Flavoprotein

Enzyme and pathway databases

BRENDAi1.5.8.4. 3474.
ReactomeiR-MMU-6798163. Choline catabolism.
UniPathwayiUPA00291; UER00433.

Names & Taxonomyi

Protein namesi
Recommended name:
Dimethylglycine dehydrogenase, mitochondrial (EC:1.5.8.4By similarity)
Alternative name(s):
ME2GLYDH
Gene namesi
Name:Dmgdh
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 13

Organism-specific databases

MGIiMGI:1921379. Dmgdh.

Subcellular locationi

  • Mitochondrion By similarity

GO - Cellular componenti

  • mitochondrial matrix Source: GO_Central
  • mitochondrion Source: MGI

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 43MitochondrionSequence analysisAdd BLAST43
ChainiPRO_000001076844 – 869Dimethylglycine dehydrogenase, mitochondrialAdd BLAST826

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei84Tele-8alpha-FAD histidineBy similarity1
Modified residuei107N6-acetyllysineCombined sources1
Modified residuei141N6-acetyllysine; alternateCombined sources1
Modified residuei141N6-succinyllysine; alternateCombined sources1
Modified residuei161N6-acetyllysineCombined sources1
Modified residuei216N6-acetyllysineCombined sources1
Modified residuei310N6-succinyllysineCombined sources1
Modified residuei312N6-succinyllysineCombined sources1
Modified residuei328N6-acetyllysineCombined sources1
Modified residuei353N6-acetyllysineCombined sources1
Modified residuei427N6-acetyllysine; alternateCombined sources1
Modified residuei427N6-succinyllysine; alternateCombined sources1
Modified residuei469N6-acetyllysine; alternateCombined sources1
Modified residuei469N6-succinyllysine; alternateCombined sources1
Modified residuei516N6-acetyllysine; alternateCombined sources1
Modified residuei516N6-succinyllysine; alternateCombined sources1
Modified residuei648N6-acetyllysine; alternateCombined sources1
Modified residuei648N6-succinyllysine; alternateCombined sources1
Modified residuei757N6-acetyllysineCombined sources1
Modified residuei786N6-acetyllysine; alternateCombined sources1
Modified residuei786N6-succinyllysine; alternateCombined sources1
Modified residuei788N6-succinyllysineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiQ9DBT9.
PaxDbiQ9DBT9.
PeptideAtlasiQ9DBT9.
PRIDEiQ9DBT9.

PTM databases

iPTMnetiQ9DBT9.
PhosphoSitePlusiQ9DBT9.
SwissPalmiQ9DBT9.

Expressioni

Gene expression databases

BgeeiENSMUSG00000042102.
CleanExiMM_DMGDH.
GenevisibleiQ9DBT9. MM.

Interactioni

Protein-protein interaction databases

IntActiQ9DBT9. 2 interactors.
MINTiMINT-1840379.
STRINGi10090.ENSMUSP00000039663.

Structurei

3D structure databases

ProteinModelPortaliQ9DBT9.
SMRiQ9DBT9.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni573 – 575Tetrahydrofolate bindingBy similarity3
Regioni676 – 678Tetrahydrofolate bindingBy similarity3

Sequence similaritiesi

Belongs to the GcvT family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2844. Eukaryota.
COG0404. LUCA.
COG0665. LUCA.
GeneTreeiENSGT00530000063120.
HOGENOMiHOG000251716.
HOVERGENiHBG081945.
InParanoidiQ9DBT9.
KOiK00315.
OMAiYESQEKM.
OrthoDBiEOG091G01OO.
PhylomeDBiQ9DBT9.
TreeFamiTF314735.

Family and domain databases

Gene3Di2.40.30.110. 1 hit.
3.30.1360.120. 2 hits.
3.50.50.60. 1 hit.
InterProiView protein in InterPro
IPR006076. FAD-dep_OxRdtase.
IPR023753. FAD/NAD-binding_dom.
IPR032503. FAO_M.
IPR013977. GCV_T_C.
IPR006222. GCV_T_N.
IPR029043. GcvT/YgfZ_C.
IPR027266. TrmE/GcvT_dom1.
PfamiView protein in Pfam
PF01266. DAO. 1 hit.
PF16350. FAO_M. 1 hit.
PF01571. GCV_T. 1 hit.
PF08669. GCV_T_C. 1 hit.
SUPFAMiSSF101790. SSF101790. 1 hit.
SSF51905. SSF51905. 2 hits.

Sequencei

Sequence statusi: Complete.

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

Q9DBT9-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLRPGALRLR GLALRGSPRR PSSAGLREGQ ESPASPPEWK DRAETVIIGG
60 70 80 90 100
GCVGVSLAYH LAKAGMRDVV LMEKSELTAG STWHAAGLTT YFHPGINLKK
110 120 130 140 150
IHYDSIKLYE RLEEETGQVV GFHQPGSIRL ATTPVRVDEF KYQMTRTNWH
160 170 180 190 200
ATEQYIIEPE KIHELFPLLN MNKILAGLYN PGDGHIDPYS LTMALAAGAR
210 220 230 240 250
KYGALLKYPA PVTSLKPRPD GTWDVETPQG SVRANRIVNA AGFWAREVGK
260 270 280 290 300
MIGLDHPLIP VQHQYVVTST IPEVKALKRE LPVLRDLEGS YYLRQERDGL
310 320 330 340 350
LFGPYESQEK MKLQASWVTH GVPPGFGKEL FESDLDRISD HLEAAMEMIP
360 370 380 390 400
VLKKADIINV VNGPITYSPD ILPMVGPHQG VRNYWVATGF GYGIIHAGGV
410 420 430 440 450
GKFLSDWILH GEPPFDLIEL DPNRYGKWTT TQYTEAKARE SYGFNNIVGY
460 470 480 490 500
PKEERFAGRP TQRVSGLYKT LKSKCSMGFH AGWEQPHWFY KPGQDTQYRP
510 520 530 540 550
SFRRTNWFEP VGSEYKQVMQ RVGVIDLSPF GKFNIKGRDS TQLLDHLFAN
560 570 580 590 600
VIPKVGFTNI SHMLTPRGRV YAELTVSQQS PGEFLLITGS GSELHDLRWI
610 620 630 640 650
EEAAFRGGYD VEIQNITDEF GVLGVAGPYA RRVLQKLTSE DLSDDAFKFL
660 670 680 690 700
QTKSFNISDI PVTAIRISYT GELGWELYHR REDSATLYER IMSAGQEEGI
710 720 730 740 750
GDFGTYALNA LRLEKAFRAW GSEMNCDTNP LEAGLEYFVK LNKPADFIGK
760 770 780 790 800
QALKQIKTEG LKRRLVCLTV ATDDVDPEGN ESIWYKGKVV GNTTSGSYSY
810 820 830 840 850
SIQKSLAFAY VPVQLSEVGQ QVEVELLGKN YPATIIQEPL VLTEPARARL
860
QKDGKKTNLE KGPSRTTKL
Length:869
Mass (Da):97,255
Last modified:June 1, 2001 - v1
Checksum:iD415C743105A0547
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti758T → A in AAH24126 (PubMed:15489334).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK004755 mRNA. Translation: BAB23536.1.
CT030023, AC131739 Genomic DNA. Translation: CAO77970.1.
BC024126 mRNA. Translation: AAH24126.1.
CCDSiCCDS26690.1.
RefSeqiNP_083048.1. NM_028772.3.
UniGeneiMm.21789.

Genome annotation databases

EnsembliENSMUST00000048001; ENSMUSP00000039663; ENSMUSG00000042102.
GeneIDi74129.
KEGGimmu:74129.
UCSCiuc007rll.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK004755 mRNA. Translation: BAB23536.1.
CT030023, AC131739 Genomic DNA. Translation: CAO77970.1.
BC024126 mRNA. Translation: AAH24126.1.
CCDSiCCDS26690.1.
RefSeqiNP_083048.1. NM_028772.3.
UniGeneiMm.21789.

3D structure databases

ProteinModelPortaliQ9DBT9.
SMRiQ9DBT9.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ9DBT9. 2 interactors.
MINTiMINT-1840379.
STRINGi10090.ENSMUSP00000039663.

PTM databases

iPTMnetiQ9DBT9.
PhosphoSitePlusiQ9DBT9.
SwissPalmiQ9DBT9.

Proteomic databases

MaxQBiQ9DBT9.
PaxDbiQ9DBT9.
PeptideAtlasiQ9DBT9.
PRIDEiQ9DBT9.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000048001; ENSMUSP00000039663; ENSMUSG00000042102.
GeneIDi74129.
KEGGimmu:74129.
UCSCiuc007rll.1. mouse.

Organism-specific databases

CTDi29958.
MGIiMGI:1921379. Dmgdh.

Phylogenomic databases

eggNOGiKOG2844. Eukaryota.
COG0404. LUCA.
COG0665. LUCA.
GeneTreeiENSGT00530000063120.
HOGENOMiHOG000251716.
HOVERGENiHBG081945.
InParanoidiQ9DBT9.
KOiK00315.
OMAiYESQEKM.
OrthoDBiEOG091G01OO.
PhylomeDBiQ9DBT9.
TreeFamiTF314735.

Enzyme and pathway databases

UniPathwayiUPA00291; UER00433.
BRENDAi1.5.8.4. 3474.
ReactomeiR-MMU-6798163. Choline catabolism.

Miscellaneous databases

PROiPR:Q9DBT9.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000042102.
CleanExiMM_DMGDH.
GenevisibleiQ9DBT9. MM.

Family and domain databases

Gene3Di2.40.30.110. 1 hit.
3.30.1360.120. 2 hits.
3.50.50.60. 1 hit.
InterProiView protein in InterPro
IPR006076. FAD-dep_OxRdtase.
IPR023753. FAD/NAD-binding_dom.
IPR032503. FAO_M.
IPR013977. GCV_T_C.
IPR006222. GCV_T_N.
IPR029043. GcvT/YgfZ_C.
IPR027266. TrmE/GcvT_dom1.
PfamiView protein in Pfam
PF01266. DAO. 1 hit.
PF16350. FAO_M. 1 hit.
PF01571. GCV_T. 1 hit.
PF08669. GCV_T_C. 1 hit.
SUPFAMiSSF101790. SSF101790. 1 hit.
SSF51905. SSF51905. 2 hits.
ProtoNetiSearch...

Entry informationi

Entry nameiM2GD_MOUSE
AccessioniPrimary (citable) accession number: Q9DBT9
Secondary accession number(s): B1B1D0, Q8R1S7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 7, 2004
Last sequence update: June 1, 2001
Last modified: April 12, 2017
This is version 121 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, 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.