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Protein

Methylmalonic aciduria type A homolog, mitochondrial

Gene

Mmaa

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

Functioni

GTPase, binds and hydrolyzes GTP. Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis. Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MUT) and reactivation of the enzyme during catalysis.By similarity

Catalytic activityi

GTP + H2O = GDP + phosphate.By similarity

Activity regulationi

GTPase activity is stimulated by MUT.By similarity

Pathwayi: adenosylcobalamin biosynthesis

This protein is involved in the pathway adenosylcobalamin biosynthesis, which is part of Cofactor biosynthesis.By similarity
View all proteins of this organism that are known to be involved in the pathway adenosylcobalamin biosynthesis and in Cofactor biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei289GTPBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi147 – 155GTPBy similarity9
Nucleotide bindingi325 – 327GTPBy similarity3

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionChaperone, Hydrolase
LigandGTP-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-MMU-196741 Cobalamin (Cbl, vitamin B12) transport and metabolism
R-MMU-71032 Propionyl-CoA catabolism
UniPathwayi
UPA00148

Names & Taxonomyi

Protein namesi
Recommended name:
Methylmalonic aciduria type A homolog, mitochondrialCurated (EC:3.6.-.-By similarity)
Gene namesi
Name:MmaaImported
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 8

Organism-specific databases

MGIiMGI:1923805 Mmaa

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 – 62MitochondrionSequence analysisAdd BLAST62
ChainiPRO_000000228663 – 415Methylmalonic aciduria type A homolog, mitochondrialAdd BLAST353

Proteomic databases

MaxQBiQ8C7H1
PaxDbiQ8C7H1
PeptideAtlasiQ8C7H1
PRIDEiQ8C7H1

PTM databases

iPTMnetiQ8C7H1
PhosphoSitePlusiQ8C7H1
SwissPalmiQ8C7H1

Expressioni

Gene expression databases

BgeeiENSMUSG00000037022 Expressed in 258 organ(s), highest expression level in heart left ventricle
ExpressionAtlasiQ8C7H1 baseline and differential
GenevisibleiQ8C7H1 MM

Interactioni

Subunit structurei

Homodimer.By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000048826

Structurei

3D structure databases

ProteinModelPortaliQ8C7H1
SMRiQ8C7H1
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiENOG410IFVS Eukaryota
COG1703 LUCA
GeneTreeiENSGT00390000009908
HOGENOMiHOG000003902
HOVERGENiHBG045588
InParanoidiQ8C7H1
KOiK07588
OMAiEAMLVCE
OrthoDBiEOG091G08RT
PhylomeDBiQ8C7H1
TreeFamiTF313243

Family and domain databases

InterProiView protein in InterPro
IPR003593 AAA+_ATPase
IPR005129 GTPase_ArgK
IPR027417 P-loop_NTPase
PANTHERiPTHR23408:SF3 PTHR23408:SF3, 1 hit
SMARTiView protein in SMART
SM00382 AAA, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
TIGRFAMsiTIGR00750 lao, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

Q8C7H1-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MTISTLLLSP NRRLLTCLSR VPSPWLLHSS HPAPGPPGAL PNCFGHHCTK
60 70 80 90 100
RVLLSDGFRR TLCVQATLKD HTEGLSDKEQ RFVDRLYTGL VKGQRACLAE
110 120 130 140 150
AITLVESTHT RKRELAQVLL QRVLALQREQ ELRNQGKPLT FRVGLSGPPG
160 170 180 190 200
AGKSTFIECF GKMLTEQGHR LSVLAVDPSS CTSGGSLLGD KTRMIELSRD
210 220 230 240 250
MNAYIRPSPT SGTLGGVTRT TNEAIVLCEG GGYDIILIET VGVGQSEFAV
260 270 280 290 300
ADMVDMFVLL LPPAGGDELQ GIKRGIIEMA DLVVITKSDG DLIVPARRIQ
310 320 330 340 350
AEYVSALKLL RRRSEVWRPK VIRISARSGE GITEMWDTMR EFQHQMLASG
360 370 380 390 400
ELAAKRQTQH KVWMWNLIQE NVLEHFKTHP SIREQIPLME RKVLSGALSP
410
GRAADLLLKA FKSRH
Length:415
Mass (Da):45,932
Last modified:March 1, 2003 - v1
Checksum:iA46967424CC4CC06
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A1B0GSL6A0A1B0GSL6_MOUSE
Methylmalonic aciduria type A homol...
Mmaa
155Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti31H → P in AAH21954 (PubMed:15489334).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK050255 mRNA Translation: BAC34149.1
BC021954 mRNA Translation: AAH21954.1
BC027398 mRNA Translation: AAH27398.1
CCDSiCCDS22436.1
RefSeqiNP_598584.2, NM_133823.4
XP_006530625.1, XM_006530562.3
XP_006530626.1, XM_006530563.3
XP_006530627.1, XM_006530564.3
XP_011246563.1, XM_011248261.2
XP_011246564.1, XM_011248262.2
XP_011246565.1, XM_011248263.2
XP_011246566.1, XM_011248264.2
XP_011246567.1, XM_011248265.2
XP_011246568.1, XM_011248266.2
XP_011246569.1, XM_011248267.2
XP_011246570.1, XM_011248268.2
XP_011246571.1, XM_011248269.2
XP_011246572.1, XM_011248270.2
XP_017167993.1, XM_017312504.1
XP_017167994.1, XM_017312505.1
UniGeneiMm.26510

Genome annotation databases

EnsembliENSMUST00000048718; ENSMUSP00000048826; ENSMUSG00000037022
GeneIDi109136
KEGGimmu:109136
UCSCiuc009mim.1 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK050255 mRNA Translation: BAC34149.1
BC021954 mRNA Translation: AAH21954.1
BC027398 mRNA Translation: AAH27398.1
CCDSiCCDS22436.1
RefSeqiNP_598584.2, NM_133823.4
XP_006530625.1, XM_006530562.3
XP_006530626.1, XM_006530563.3
XP_006530627.1, XM_006530564.3
XP_011246563.1, XM_011248261.2
XP_011246564.1, XM_011248262.2
XP_011246565.1, XM_011248263.2
XP_011246566.1, XM_011248264.2
XP_011246567.1, XM_011248265.2
XP_011246568.1, XM_011248266.2
XP_011246569.1, XM_011248267.2
XP_011246570.1, XM_011248268.2
XP_011246571.1, XM_011248269.2
XP_011246572.1, XM_011248270.2
XP_017167993.1, XM_017312504.1
XP_017167994.1, XM_017312505.1
UniGeneiMm.26510

3D structure databases

ProteinModelPortaliQ8C7H1
SMRiQ8C7H1
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000048826

PTM databases

iPTMnetiQ8C7H1
PhosphoSitePlusiQ8C7H1
SwissPalmiQ8C7H1

Proteomic databases

MaxQBiQ8C7H1
PaxDbiQ8C7H1
PeptideAtlasiQ8C7H1
PRIDEiQ8C7H1

Protocols and materials databases

DNASUi109136
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000048718; ENSMUSP00000048826; ENSMUSG00000037022
GeneIDi109136
KEGGimmu:109136
UCSCiuc009mim.1 mouse

Organism-specific databases

CTDi166785
MGIiMGI:1923805 Mmaa

Phylogenomic databases

eggNOGiENOG410IFVS Eukaryota
COG1703 LUCA
GeneTreeiENSGT00390000009908
HOGENOMiHOG000003902
HOVERGENiHBG045588
InParanoidiQ8C7H1
KOiK07588
OMAiEAMLVCE
OrthoDBiEOG091G08RT
PhylomeDBiQ8C7H1
TreeFamiTF313243

Enzyme and pathway databases

UniPathwayi
UPA00148

ReactomeiR-MMU-196741 Cobalamin (Cbl, vitamin B12) transport and metabolism
R-MMU-71032 Propionyl-CoA catabolism

Miscellaneous databases

PROiPR:Q8C7H1
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000037022 Expressed in 258 organ(s), highest expression level in heart left ventricle
ExpressionAtlasiQ8C7H1 baseline and differential
GenevisibleiQ8C7H1 MM

Family and domain databases

InterProiView protein in InterPro
IPR003593 AAA+_ATPase
IPR005129 GTPase_ArgK
IPR027417 P-loop_NTPase
PANTHERiPTHR23408:SF3 PTHR23408:SF3, 1 hit
SMARTiView protein in SMART
SM00382 AAA, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
TIGRFAMsiTIGR00750 lao, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiMMAA_MOUSE
AccessioniPrimary (citable) accession number: Q8C7H1
Secondary accession number(s): Q8R2N3, Q8VC22
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 7, 2003
Last sequence update: March 1, 2003
Last modified: November 7, 2018
This is version 127 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. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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