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Q811U4

- MFN1_MOUSE

UniProt

Q811U4 - MFN1_MOUSE

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Protein
Mitofusin-1
Gene
Mfn1
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 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 – 898GTP Inferred
Nucleotide bindingi178 – 1825GTP Inferred
Nucleotide bindingi237 – 2404GTP Inferred

GO - Molecular functioni

  1. GTP binding Source: UniProtKB-KW
  2. GTPase activity Source: InterPro
  3. protein binding Source: IntAct

GO - Biological processi

  1. mitochondrial fusion Source: MGI
  2. multicellular organismal development Source: UniProtKB-KW
Complete GO annotation...

Keywords - Molecular functioni

Developmental protein, Hydrolase

Keywords - Ligandi

GTP-binding, Nucleotide-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Mitofusin-1 (EC:3.6.5.-)
Alternative name(s):
Transmembrane GTPase MFN1
Gene namesi
Name:Mfn1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 3

Organism-specific databases

MGIiMGI:1914664. Mfn1.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 584584Cytoplasmic Reviewed prediction
Add
BLAST
Transmembranei585 – 60521Helical; Name=1; Reviewed prediction
Add
BLAST
Topological domaini606 – 6083Mitochondrial intermembrane Reviewed prediction
Transmembranei609 – 62921Helical; Name=2; Reviewed prediction
Add
BLAST
Topological domaini630 – 741112Cytoplasmic Reviewed prediction
Add
BLAST

GO - Cellular componenti

  1. integral component of membrane Source: UniProtKB-KW
  2. mitochondrial outer membrane Source: UniProtKB-SubCell
  3. mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechi

Disruption phenotypei

In cardiomyocytes, no effect on mitochondrial morphometry or respiratory function.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 741741Mitofusin-1
PRO_0000127673Add
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 (1 Publication).

Keywords - PTMi

Ubl conjugation

Proteomic databases

MaxQBiQ811U4.
PaxDbiQ811U4.
PRIDEiQ811U4.

PTM databases

PhosphoSiteiQ811U4.

Expressioni

Tissue specificityi

Widely expressed.1 Publication

Developmental stagei

Expressed in E8.5, E9.5, E10.5 and E11.5 embryos.1 Publication

Gene expression databases

ArrayExpressiQ811U4.
BgeeiQ811U4.
CleanExiMM_MFN1.
GenevestigatoriQ811U4.

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 By similarity.2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
LRRK2Q5S0073EBI-9029118,EBI-5323863From a different organism.

Protein-protein interaction databases

BioGridi212170. 2 interactions.
IntActiQ811U4. 1 interaction.

Structurei

Secondary structure

Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Helixi676 – 73358

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1T3JX-ray2.50A660-735[»]
ProteinModelPortaliQ811U4.
SMRiQ811U4. Positions 674-735.

Miscellaneous databases

EvolutionaryTraceiQ811U4.

Family & Domainsi

Domains and Repeats

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

Coiled coil

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Coiled coili371 – 408381 Publication
Add
BLAST
Coiled coili677 – 735591 Publication
Add
BLAST

Sequence similaritiesi

Keywords - Domaini

Coiled coil, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiCOG0699.
GeneTreeiENSGT00390000013727.
HOVERGENiHBG052465.
InParanoidiQ3URC4.
KOiK06030.
OMAiAMTDEIC.
OrthoDBiEOG7HB58M.
TreeFamiTF314289.

Family and domain databases

Gene3Di3.40.50.300. 2 hits.
InterProiIPR001401. Dynamin_GTPase.
IPR006884. Fzo/mitofusin_HR2.
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.

Q811U4-1 [UniParc]FASTAAdd to Basket

« Hide

MAETVSPLKH FVLAKKAITA IFGQLLEFVT EGSHFVEATY RNPELDRIAS    50
EDDLVEIQGY RNKLAVIGEV LSRRHMKVAF FGRTSSGKSS VINAMLWDKV 100
LPSGIGHTTN CFLSVEGTDG DKAYLMTEGS DEKKSVKTVN QLAHALHMDK 150
DLKAGCLVHV FWPKAKCALL RDDLVLVDSP GTDVTTELDI WIDKFCLDAD 200
VFVLVANSES TLMNTEKHFF HKVNERLSKP NIFILNNRWD ASASEPEYME 250
DVRRQHMERC LHFLVEELKV VSPSEARNRI FFVSAKEVLN SRKHKAQGMP 300
EGGGALAEGF QARLQEFQNF EQTFEECISQ SAVKTKFEQH TIRAKQILDT 350
VKNILDSVNV AAAEKRVYSM EEREDQIDRL DFIRNQMNLL TLDVKKKIKE 400
VTEEVANKVS CAMTDEICRL SVLVDEFCSE FHPTPSVLKV YKSELNKHIE 450
DGMGRNLADR CTNEVNASIL QSQQEIIENL KPLLPAGIQN KLHTLIPCKK 500
FDLSYDLNCH KLCSDFQEDI VFRFSLGWSS LVHRFLGSTN AQRVLLGLSE 550
PIFQVPRSLA STPTAPSNPA APDNAAQEEL MITLITGLAS LTSRTSMGII 600
VVGGVIWKTV GWKLISVTLS MYGALYLYER LTWTTRAKER AFKQQFVNYA 650
TEKLQMIVSF TSANCSHQVQ QEMATTFARL CQQVDVTQKH LEEEIARLSK 700
EIDQLEKIQN NSKLLRNKAV QLESELENFS KQFLHPSSGE S 741
Length:741
Mass (Da):83,726
Last modified:July 27, 2011 - v3
Checksum:i36E5E90DB0C0D5A1
GO

Sequence cautioni

The sequence AAH47050.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.
The sequence BAC25260.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti209 – 2091E → G in BAB31111. 1 Publication
Sequence conflicti328 – 3292IS → VL in BAB31111. 1 Publication
Sequence conflicti335 – 3351T → A in BAB31111. 1 Publication
Sequence conflicti521 – 5211V → A in BAB31111. 1 Publication
Sequence conflicti588 – 5881L → W in BAB31111. 1 Publication
Sequence conflicti619 – 6191L → S in BAB31111. 1 Publication
Sequence conflicti718 – 7181K → R in BAB31111. 1 Publication
Sequence conflicti720 – 7201V → I in AAH02133. 1 Publication
Sequence conflicti720 – 7201V → I in AAH56641. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AY174062 mRNA. Translation: AAO34660.1.
AK009490 mRNA. Translation: BAC25260.1. Different initiation.
AK018181 mRNA. Translation: BAB31111.1.
AK141611 mRNA. Translation: BAE24764.1.
BC002133 mRNA. Translation: AAH02133.1.
BC047050 mRNA. Translation: AAH47050.1. Different initiation.
BC056641 mRNA. Translation: AAH56641.1.
CCDSiCCDS17296.1.
RefSeqiNP_077162.2. NM_024200.4.
UniGeneiMm.290414.

Genome annotation databases

EnsembliENSMUST00000091257; ENSMUSP00000088801; ENSMUSG00000027668.
ENSMUST00000118286; ENSMUSP00000113251; ENSMUSG00000027668.
GeneIDi67414.
KEGGimmu:67414.
UCSCiuc008owi.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AY174062 mRNA. Translation: AAO34660.1 .
AK009490 mRNA. Translation: BAC25260.1 . Different initiation.
AK018181 mRNA. Translation: BAB31111.1 .
AK141611 mRNA. Translation: BAE24764.1 .
BC002133 mRNA. Translation: AAH02133.1 .
BC047050 mRNA. Translation: AAH47050.1 . Different initiation.
BC056641 mRNA. Translation: AAH56641.1 .
CCDSi CCDS17296.1.
RefSeqi NP_077162.2. NM_024200.4.
UniGenei Mm.290414.

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
Entry Method Resolution (Å) Chain Positions PDBsum
1T3J X-ray 2.50 A 660-735 [» ]
ProteinModelPortali Q811U4.
SMRi Q811U4. Positions 674-735.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 212170. 2 interactions.
IntActi Q811U4. 1 interaction.

PTM databases

PhosphoSitei Q811U4.

Proteomic databases

MaxQBi Q811U4.
PaxDbi Q811U4.
PRIDEi Q811U4.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000091257 ; ENSMUSP00000088801 ; ENSMUSG00000027668 .
ENSMUST00000118286 ; ENSMUSP00000113251 ; ENSMUSG00000027668 .
GeneIDi 67414.
KEGGi mmu:67414.
UCSCi uc008owi.2. mouse.

Organism-specific databases

CTDi 55669.
MGIi MGI:1914664. Mfn1.

Phylogenomic databases

eggNOGi COG0699.
GeneTreei ENSGT00390000013727.
HOVERGENi HBG052465.
InParanoidi Q3URC4.
KOi K06030.
OMAi AMTDEIC.
OrthoDBi EOG7HB58M.
TreeFami TF314289.

Miscellaneous databases

ChiTaRSi MFN1. mouse.
EvolutionaryTracei Q811U4.
NextBioi 324498.
PROi Q811U4.
SOURCEi Search...

Gene expression databases

ArrayExpressi Q811U4.
Bgeei Q811U4.
CleanExi MM_MFN1.
Genevestigatori Q811U4.

Family and domain databases

Gene3Di 3.40.50.300. 2 hits.
InterProi IPR001401. Dynamin_GTPase.
IPR006884. Fzo/mitofusin_HR2.
IPR027088. Mitofusin-1.
IPR027094. Mitofusin_fam.
IPR027417. P-loop_NTPase.
[Graphical view ]
PANTHERi PTHR10465. PTHR10465. 1 hit.
PTHR10465:SF2. PTHR10465:SF2. 1 hit.
Pfami PF00350. Dynamin_N. 1 hit.
PF04799. Fzo_mitofusin. 1 hit.
[Graphical view ]
SUPFAMi SSF52540. SSF52540. 1 hit.
PROSITEi PS51718. G_DYNAMIN_2. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development."
    Chen H., Detmer S.A., Ewald A.J., Griffin E.E., Fraser S.E., Chan D.C.
    J. Cell Biol. 160:189-200(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA], FUNCTION, DEVELOPMENTAL STAGE, MULTIMERIZATION.
    Strain: FVB.
  2. "The transcriptional landscape of the mammalian genome."
    Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
    , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
    Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: C57BL/6J.
    Tissue: Hippocampus, Medulla oblongata and Tongue.
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: FVB/N.
    Tissue: Liver and Mammary tumor.
  4. "Membrane topology and mitochondrial targeting of mitofusins, ubiquitous mammalian homologs of the transmembrane GTPase Fzo."
    Rojo M., Legros F., Chateau D., Lombes A.
    J. Cell Sci. 115:1663-1674(2002) [PubMed] [Europe PMC] [Abstract]
    Cited for: TISSUE SPECIFICITY.
  5. "PINK1-phosphorylated mitofusin 2 is a Parkin receptor for culling damaged mitochondria."
    Chen Y., Dorn G.W. II
    Science 340:471-475(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: DISRUPTION PHENOTYPE.
  6. "A small natural molecule promotes mitochondrial fusion through inhibition of the deubiquitinase USP30."
    Yue W., Chen Z., Liu H., Yan C., Chen M., Feng D., Yan C., Wu H., Du L., Wang Y., Liu J., Huang X., Xia L., Liu L., Wang X., Jin H., Wang J., Song Z., Hao X., Chen Q.
    Cell Res. 24:482-496(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: UBIQUITINATION, DEUBIQUITINATION.
  7. "Structural basis of mitochondrial tethering by mitofusin complexes."
    Koshiba T., Detmer S.A., Kaiser J.T., Chen H., McCaffery J.M., Chan D.C.
    Science 305:858-862(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: X-RAY CRYSTALLOGRAPHY (2.5 ANGSTROMS) OF 660-735, FUNCTION, SUBUNIT, COILED-COIL DOMAINS.

Entry informationi

Entry nameiMFN1_MOUSE
AccessioniPrimary (citable) accession number: Q811U4
Secondary accession number(s): Q3URC4
, Q811D5, Q8CEY6, Q99M10, Q9D395
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 24, 2004
Last sequence update: July 27, 2011
Last modified: September 3, 2014
This is version 101 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Miscellaneous

MFN1 deficient mice die early during embryonic development, due to altered mitochondria morphology, which are fragmented, showing that mitochondrial fusion is essential for embryonic development.

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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