Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Mitofusin-1

Gene

Mfn1

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

Essential transmembrane GTPase, which mediates mitochondrial fusion. Fusion of mitochondria occurs in many cell types and constitutes an important step in mitochondria morphology, which is balanced between fusion and fission. MFN1 acts independently of the cytoskeleton. Overexpression induces the formation of mitochondrial networks.2 Publications

Catalytic activityi

GTP + H2O = GDP + phosphate.

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi82 – 898GTPCurated
Nucleotide bindingi178 – 1825GTPCurated
Nucleotide bindingi237 – 2404GTPCurated

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Ligandi

GTP-binding, Nucleotide-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Mitofusin-1 (EC:3.6.5.-)
Alternative name(s):
Mitochondrial transmembrane GTPase FZO1B
Transmembrane GTPase MFN1
Gene namesi
Name:Mfn1
Synonyms:Fzo1b
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
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)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 584584CytoplasmicSequence analysisAdd
BLAST
Transmembranei585 – 60521Helical; Name=1Sequence analysisAdd
BLAST
Topological domaini606 – 6083Mitochondrial intermembraneSequence analysis
Transmembranei609 – 62921Helical; Name=2Sequence analysisAdd
BLAST
Topological domaini630 – 741112CytoplasmicSequence analysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi88 – 881K → T: Induces partial mitochondria fragmentation; when overexpressed. 1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 741741Mitofusin-1PRO_0000127674Add
BLAST

Post-translational modificationi

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 PARK2. Ubiquitinated by non-degradative ubiquitin by PARK2, promoting mitochondrial fusion; deubiquitination by USP30 inhibits mitochondrial fusion (By similarity).By similarity

Keywords - PTMi

Ubl conjugation

Proteomic databases

PaxDbiQ8R4Z9.
PRIDEiQ8R4Z9.

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

Forms homomultimers and heteromultimers with MFN2. Multimerization, which is mediated by the second coiled coil region, may play an essential role in mitochondrion fusion. Participates in a high molecular weight multiprotein complex. Interacts with VAT1.1 Publication

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000060265.

Structurei

3D structure databases

ProteinModelPortaliQ8R4Z9.
SMRiQ8R4Z9. Positions 674-735.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini72 – 321250Dynamin-type GAdd
BLAST

Coiled coil

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Coiled coili371 – 40838Sequence analysisAdd
BLAST
Coiled coili677 – 73559Sequence analysisAdd
BLAST

Sequence similaritiesi

Keywords - Domaini

Coiled coil, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0448. Eukaryota.
COG0699. LUCA.
HOVERGENiHBG052465.
InParanoidiQ8R4Z9.
KOiK06030.
PhylomeDBiQ8R4Z9.

Family and domain databases

Gene3Di3.40.50.300. 2 hits.
InterProiIPR022812. Dynamin_SF.
IPR006884. Fzo/mitofusin_HR2.
IPR030381. G_DYNAMIN_dom.
IPR027088. Mitofusin-1.
IPR027094. Mitofusin_fam.
IPR027417. P-loop_NTPase.
[Graphical view]
PANTHERiPTHR10465. PTHR10465. 1 hit.
PTHR10465:SF2. PTHR10465:SF2. 1 hit.
PfamiPF00350. Dynamin_N. 1 hit.
PF04799. Fzo_mitofusin. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
PROSITEiPS51718. G_DYNAMIN_2. 1 hit.
[Graphical view]

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.

Cross-referencesi

Sequence databases

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

3D structure databases

ProteinModelPortaliQ8R4Z9.
SMRiQ8R4Z9. Positions 674-735.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000060265.

Proteomic databases

PaxDbiQ8R4Z9.
PRIDEiQ8R4Z9.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi55669.
RGDi621460. Mfn1.

Phylogenomic databases

eggNOGiKOG0448. Eukaryota.
COG0699. LUCA.
HOVERGENiHBG052465.
InParanoidiQ8R4Z9.
KOiK06030.
PhylomeDBiQ8R4Z9.

Miscellaneous databases

NextBioi623077.
PROiQ8R4Z9.

Family and domain databases

Gene3Di3.40.50.300. 2 hits.
InterProiIPR022812. Dynamin_SF.
IPR006884. Fzo/mitofusin_HR2.
IPR030381. G_DYNAMIN_dom.
IPR027088. Mitofusin-1.
IPR027094. Mitofusin_fam.
IPR027417. P-loop_NTPase.
[Graphical view]
PANTHERiPTHR10465. PTHR10465. 1 hit.
PTHR10465:SF2. PTHR10465:SF2. 1 hit.
PfamiPF00350. Dynamin_N. 1 hit.
PF04799. Fzo_mitofusin. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
PROSITEiPS51718. G_DYNAMIN_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

  1. "Two mitofusin proteins, mammalian homologues of FZO, with distinct functions are both required for mitochondrial fusion."
    Eura Y., Ishihara N., Yokota S., Mihara K.
    J. Biochem. 134:333-344(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA], FUNCTION, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, MULTIMERIZATION, MUTAGENESIS OF LYS-88.
    Tissue: Liver.
  2. "Regulation of mitochondrial morphology by membrane potential, and DRP1-dependent division and FZO1-dependent fusion reaction in mammalian cells."
    Ishihara N., Jofuku A., Eura Y., Mihara K.
    Biochem. Biophys. Res. Commun. 301:891-898(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION.
  3. "Stage-specific enhanced expression of mitochondrial fusion and fission factors during spermatogenesis in rat testis."
    Honda S., Hirose S.
    Biochem. Biophys. Res. Commun. 311:424-432(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: TISSUE SPECIFICITY.
  4. "Identification of a novel protein that regulates mitochondrial fusion by modulating mitofusin (Mfn) protein function."
    Eura Y., Ishihara N., Oka T., Mihara K.
    J. Cell Sci. 119:4913-4925(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH VAT1.

Entry informationi

Entry nameiMFN1_RAT
AccessioniPrimary (citable) accession number: Q8R4Z9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 24, 2004
Last sequence update: June 1, 2002
Last modified: December 9, 2015
This is version 78 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, Reference proteome

Documents

  1. 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.