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Protein

Mitofusin-1

Gene

Mfn1

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Mitochondrial outer membrane GTPase that mediates mitochondrial clustering and fusion (PubMed:14561718, PubMed:12589796). Membrane clustering requires GTPase activity. It may involve a major rearrangement of the coiled coil domains (By similarity). Mitochondria are highly dynamic organelles, and their morphology is determined by the equilibrium between mitochondrial fusion and fission events (PubMed:14561718). Overexpression induces the formation of mitochondrial networks (in vitro) (PubMed:14561718). Has low GTPase activity (By similarity).By similarity2 Publications

Catalytic activityi

GTP + H2O = GDP + phosphate.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei284GTPBy similarity1
Binding sitei286GTPBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi85 – 90GTPBy similarity6
Nucleotide bindingi237 – 240GTPBy similarity4

GO - Molecular functioni

GO - Biological processi

  • cellular response to oxygen-glucose deprivation Source: RGD
  • GTP metabolic process Source: UniProtKB
  • intracellular distribution of mitochondria Source: RGD
  • mitochondrial fusion Source: UniProtKB
  • mitochondrial membrane fusion Source: UniProtKB
  • mitochondrion localization Source: UniProtKB
  • mitochondrion morphogenesis Source: RGD
  • negative regulation of mitochondrial fission Source: RGD
  • positive regulation of dendritic spine morphogenesis Source: RGD
  • response to flavonoid Source: RGD
  • response to hypobaric hypoxia Source: RGD
  • response to muscle activity Source: RGD

Keywordsi

Molecular functionHydrolase
LigandGTP-binding, Nucleotide-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Mitofusin-1 (EC:3.6.5.-By similarity)
Alternative name(s):
Mitochondrial transmembrane GTPase FZO1B
Transmembrane GTPase MFN1
Gene namesi
Name:Mfn1
Synonyms:Fzo1b
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi621460. Mfn1.

Subcellular locationi

  • Mitochondrion outer membrane 1 Publication; Multi-pass membrane protein 1 Publication

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 584CytoplasmicSequence analysisAdd BLAST584
Transmembranei585 – 605Helical; Name=1Sequence analysisAdd BLAST21
Topological domaini606 – 608Mitochondrial intermembraneSequence analysis3
Transmembranei609 – 629Helical; Name=2Sequence analysisAdd BLAST21
Topological domaini630 – 741CytoplasmicSequence analysisAdd BLAST112

GO - Cellular componenti

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi88K → T: Induces partial mitochondria fragmentation; when overexpressed. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001276741 – 741Mitofusin-1Add BLAST741

Post-translational modificationi

Ubiquitinated by non-degradative ubiquitin by PRKN (By similarity). Deubiquitination by USP30 inhibits mitochondrial fusion (By similarity). Ubiquitinated by MARCH5. When mitochondria are depolarized and dysfunctional, it is ubiquitinated by a SCF (SKP1-CUL1-F-box protein) E3 ubiquitin-protein ligase complex that contains FBXO7 and PRKN (By similarity).By similarity

Keywords - PTMi

Ubl conjugation

Proteomic databases

PaxDbiQ8R4Z9.
PRIDEiQ8R4Z9.

PTM databases

iPTMnetiQ8R4Z9.
PhosphoSitePlusiQ8R4Z9.

Expressioni

Tissue specificityi

Ubiquitous. In brain, it is expressed at weaker level than MFN2, while it is expressed at a higher level than MFN2 in heart and testis. Expressed at high level in elongating spermatids of seminiferous tubules.2 Publications

Interactioni

Subunit structurei

Homodimer, also in the absence of bound GTP. Forms higher oligomers in the presence of a transition state GTP analog (By similarity). Forms homomultimers and heteromultimers with MFN2 (PubMed:14561718). Oligomerization is essential for mitochondrion fusion. Component of a high molecular weight multiprotein complex (By similarity). Interacts with VAT1 (PubMed:17105775).By similarity2 Publications

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000060265.

Structurei

3D structure databases

ProteinModelPortaliQ8R4Z9.
SMRiQ8R4Z9.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini72 – 321Dynamin-type GAdd BLAST250

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni9 – 73Part of a helix bundle domain, formed by helices from N-terminal and C-terminal regionsBy similarityAdd BLAST65
Regioni338 – 364Part of a helix bundle domain, formed by helices from N-terminal and C-terminal regionsBy similarityAdd BLAST27
Regioni703 – 734Part of a helix bundle domain, formed by helices from N-terminal and C-terminal regionsBy similarityAdd BLAST32

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili371 – 408Sequence analysisAdd BLAST38
Coiled coili677 – 735Sequence analysisAdd BLAST59

Domaini

A helix bundle is formed by helices from the N-terminal and the C-terminal part of the protein. The GTPase domain cannot be expressed by itself, without the helix bundle. Rearrangement of the helix bundle and/or of the coiled coil domains may bring membranes from adjacent mitochondria into close contact, and thereby play a role in mitochondrial fusion.By similarity

Sequence similaritiesi

Keywords - Domaini

Coiled coil, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0448. Eukaryota.
COG0699. LUCA.
HOVERGENiHBG052465.
InParanoidiQ8R4Z9.
KOiK21356.
PhylomeDBiQ8R4Z9.

Family and domain databases

InterProiView protein in InterPro
IPR022812. Dynamin_SF.
IPR006884. Fzo/mitofusin_HR2.
IPR030381. G_DYNAMIN_dom.
IPR027088. Mitofusin-1.
IPR027417. P-loop_NTPase.
PANTHERiPTHR10465:SF7. PTHR10465:SF7. 1 hit.
PfamiView protein in Pfam
PF00350. Dynamin_N. 1 hit.
PF04799. Fzo_mitofusin. 1 hit.
SUPFAMiSSF52540. SSF52540. 1 hit.
PROSITEiView protein in PROSITE
PS51718. G_DYNAMIN_2. 1 hit.

Sequencei

Sequence statusi: Complete.

Q8R4Z9-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAETVSPLKH FVLAKKAITA IFGQLLEFVT EGSHFVEATY RNPELDRIAT
60 70 80 90 100
EDDLVEIQGY RNKLAVIGEV LSRRHMKVAF FGRTSSGKSS VINAMLWDKV
110 120 130 140 150
LPSGIGHTTN CFLSVEGTDG DKAYLMTEGS DEKKSVKTVN QLAHALHMDK
160 170 180 190 200
DLKAGCLVHV FWPKAKCALL RDDLVLVDSP GTDVTTELDI WIDKFCLDAD
210 220 230 240 250
VFVLVANSES TLMNTEKHFF HKVNERLSKP NIFILNNRWD ASASEPEYME
260 270 280 290 300
DVRRQHMERC LHFLVEELKV VSPLEARNRI FFVSAKEVLN SRMNKAQGMP
310 320 330 340 350
EGGGALAEGF QARLQEFQNF EQTFEECISQ SAVKTKFEQH TIRAKQILDT
360 370 380 390 400
VKNILDSVNV AAAEKRVYSM EEREDQIDRL DFIRNQMNLL TMDVKKKIKE
410 420 430 440 450
VTEEVANKVS CAMTDEICRL SVLVDEFCSE FHPTPSVLKV YKSELNKHIE
460 470 480 490 500
DGMGRNLADR CTSEVNASIL QSQQEIIENL KPLLPAGIQN KLHTLIPCKK
510 520 530 540 550
FDLSYDLNCH KLCSDFQEDI VFRFSLGWSS LVHRFLGSTN AQRVLLGLSE
560 570 580 590 600
PIFQVPRSLA STPTAPSNPA APDNAAQEEL MITLITGLAS LTSRTSMGII
610 620 630 640 650
VVGGVIWKTV GWKLISVTLS MYGALYLYER LTWTTRAKER AFKQQFVNYA
660 670 680 690 700
TEKLQMIVKF TSANCSHQVQ QEMATTFARL CQQVDVTQKH LEEEIARLSK
710 720 730 740
EIDQLEKIQN NSKLLRNKAV QLERELENFS KQFLHPSSGE S
Length:741
Mass (Da):83,847
Last modified:June 1, 2002 - v1
Checksum:i39ED084CDD5D27A3
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB084166 mRNA. Translation: BAB90983.1.
RefSeqiNP_620432.1. NM_138976.1.
UniGeneiRn.160939.

Genome annotation databases

GeneIDi192647.
KEGGirno:192647.
UCSCiRGD:621460. rat.

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.

Entry informationi

Entry nameiMFN1_RAT
AccessioniPrimary (citable) accession number: Q8R4Z9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 24, 2004
Last sequence update: June 1, 2002
Last modified: May 10, 2017
This is version 86 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