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MOUSE:MFN1

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Species (Taxon ID) Mus musculus (Mouse). (10090)
Gene Name(s) Mfn1
Protein Name(s) Mitofusin-1

Transmembrane GTPase MFN1

External Links
UniProt Q811U4
EMBL AY174062
AK009490
AK018181
AK141611
BC002133
BC047050
BC056641
CCDS CCDS17296.1
RefSeq NP_077162.2
UniGene Mm.290414
PDB 1T3J
PDBsum 1T3J
ProteinModelPortal Q811U4
SMR Q811U4
BioGrid 212170
DIP DIP-60969N
IntAct Q811U4
STRING 10090.ENSMUSP00000088801
iPTMnet Q811U4
PhosphoSitePlus Q811U4
EPD Q811U4
MaxQB Q811U4
PaxDb Q811U4
PeptideAtlas Q811U4
PRIDE Q811U4
Ensembl ENSMUST00000091257
ENSMUST00000118286
GeneID 67414
KEGG mmu:67414
UCSC uc008owi.2
CTD 55669
MGI MGI:1914664
eggNOG KOG0448
COG0699
GeneTree ENSGT00390000013727
HOVERGEN HBG052465
InParanoid Q811U4
KO K06030
OMA AMTDEIC
OrthoDB EOG091G02ES
TreeFam TF314289
Reactome [www.reactome.org/content/detail/R-MMU-5205685 R-MMU-5205685]
ChiTaRS Mfn1
EvolutionaryTrace Q811U4
PRO PR:Q811U4
Proteomes UP000000589
Bgee ENSMUSG00000027668
CleanEx MM_MFN1
ExpressionAtlas Q811U4
Genevisible Q811U4
GO GO:0016021
GO:0031306
GO:0005739
GO:0005525
GO:0003924
GO:0008053
GO:0051646
GO:0007275
Gene3D 3.40.50.300
InterPro IPR022812
IPR006884
IPR030381
IPR027088
IPR027417
PANTHER PTHR10465:SF7
Pfam PF00350
PF04799
SUPFAM SSF52540
PROSITE PS51718

Annotations

Qualifier GO ID GO term name Reference ECO ID ECO term name with/from Aspect Extension Notes Status
GO:0008053

mitochondrial fusion

PMID:15899901[1]

ECO:0000315

P

Mfn1 clearly demonstrates positive regulation amount of mitochondrial fusion, as seen and inferred from Table 1 Uniprot Nomenclature: Mitofusin-1 (MFN1_MOUSE) Uniprot ID: Q811U4 Organism: Mus musculus (Mouse)

complete
CACAO 12464

GO:0010918

positive regulation of mitochondrial membrane potential

PMID:15899901[1]

ECO:0000316

UniProtKB:Q80U63


P

MFn1 reveals that it is a positive regulator of mitochondrial membrane potential as loss of membrane potential was reversible when MFN-null cells when they began to over express Mfn1. Figure 4A-C Uniprot Nomenclature: Mitofusin-1 (MFN1_MOUSE) Organism: Mus musculus (Mouse)

"Consistent with our previous study (3), we found that both Mfn1-null and Mfn2-null cells have mild heterogeneity in mitochondrial ΔΨ.2 Most Mfn1-null cells showed some mitochondria with weak MitoTracker Red staining, but this was restricted to only a few mitochondria (1–10/cell). With Mfn2-null cells, only a minority of cells showed heterogeneity in MitoTracker staining, and again this defect was restricted to a few mitochondria.

In contrast, 100% of Mfn-dm cells showed widespread heterogeneity of mitochondrial ΔΨ, with a large fraction of the mitochondrial population failing to stain well with MitoTracker Red (Fig. 4, A–C). The loss of mitochondrial ΔΨ was reversible with complete restoration of ΔΨ in Mfn-null cells overexpressing Mfn1.2 Depletion of OPA1 by RNAi similarly led to wide-spread loss of mitochondrial ΔΨ (Fig. 4, D–F). Cells overexpressing OPA1 showed no ΔΨ defects (Fig. 4, G–I). Taken together with the PEG fusion data, these results indicate that the severity of mitochondrial ΔΨ loss correlates with the severity of fusion deficiency. "

complete
CACAO 12467

GO:0030307

positive regulation of cell growth

PMID:15899901[1]

ECO:0000315

P

Mfn1 is needed for cell growth, as seen in Supplemental Fig. S1 and as Mfn2-null cells depleted of Mfn1 by shRNA grew to only 18% of the parental Mfn2-null control. Figure 3B also showed growth rescue by reintroduction of Mfn1. Uniprot Nomenclature: Mitofusin-1 (MFN1_MOUSE) Uniprot ID: Q811U4 Organism: Mus musculus (Mouse)

complete
CACAO 12468

GO:0070584

mitochondrion morphogenesis

PMID:15899901[1]

ECO:0000316

UniProtKB:Q80U63


P

Mfn-dm (double mutant) cells revealed the necessity of Mfn1 and Mfn2 for mitochondrial morphology, Therefore through this double mutant, the reliance of Mfn2 and Mfn1 for Mitochondrial morphology is confirmed. Seen in FIG. 1. Mitofusin-2 and Mitofusin-1 Mus musculus (Mouse)

complete
CACAO 12711

GO:0008053

mitochondrial fusion

PMID:15899901[1]

ECO:0000316

UniProtKB:Q80U63


P

Mfn-dm (double mutant) cells had 0% mitochondrial fusion, as seen on Table I. Table 1 and FIG. 2 illustrate the importance of both Mfn1 and Mfn2 for mitochondrial fusion. Mitofusin-2 and Mitofusin-1 Mus musculus (Mouse)

complete
CACAO 12712

GO:0016049

cell growth

PMID:15899901[1]

ECO:0000316

UniProtKB:Q80U63


P

Mfn-dm (double mutant) cells showed growth defects, as seen on FIG. 3. Thus, Mfn1 and Mfn2 are required for appropriate cell growth. Mitofusin-2 and Mitofusin-1 Mus musculus (Mouse)

complete
CACAO 12713

GO:0009060

aerobic respiration

PMID:15899901[1]

ECO:0000316

UniProtKB:Q80U63


P

Mfn-dm (double mutant) showed respiratory defects, as seen on FIG. 5. These defects were reversible, as seen in Mfn1 and Mfn2, thus showing the importance of Mfn1 and 2 for aerobic respiration. Mitofusin-2 and Mitofusin-1 Mus musculus (Mouse)

complete
CACAO 12714

part_of

GO:0098799

outer mitochondrial membrane protein complex

GO_REF:0000024

ECO:0000250

sequence similarity evidence used in manual assertion

UniProtKB:Q8IWA4

C

Seeded From UniProt

complete

part_of

GO:0031307

integral component of mitochondrial outer membrane

GO_REF:0000024

ECO:0000250

sequence similarity evidence used in manual assertion

UniProtKB:Q8IWA4

C

Seeded From UniProt

complete

enables

GO:0003924

GTPase activity

GO_REF:0000024

ECO:0000250

sequence similarity evidence used in manual assertion

UniProtKB:Q8IWA4

F

Seeded From UniProt

complete

involved_in

GO:1990613

mitochondrial membrane fusion

GO_REF:0000024

ECO:0000250

sequence similarity evidence used in manual assertion

UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

involved_in

GO:0051646

mitochondrion localization

GO_REF:0000024

ECO:0000250

sequence similarity evidence used in manual assertion

UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

involved_in

GO:0046039

GTP metabolic process

GO_REF:0000024

ECO:0000250

sequence similarity evidence used in manual assertion

UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

involved_in

GO:0008053

mitochondrial fusion

PMID:24513856[2]

ECO:0000315

mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0051646

mitochondrion localization

PMID:21873635[3]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000825684
UniProtKB:O95140
UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

part_of

GO:0031306

intrinsic component of mitochondrial outer membrane

PMID:21873635[3]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000825684
RGD:628843

C

Seeded From UniProt

complete

involved_in

GO:0008053

mitochondrial fusion

PMID:21873635[3]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

FB:FBgn0011596
FB:FBgn0029870
MGI:MGI:1914664
MGI:MGI:2442230
PANTHER:PTN000825684
RGD:628843
UniProtKB:O95140
UniProtKB:Q8IWA4
WB:WBGene00001509

P

Seeded From UniProt

complete

enables

GO:0003924

GTPase activity

PMID:21873635[3]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000049197
SGD:S000000383
UniProtKB:Q8IWA4

F

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:1990613

mitochondrial membrane fusion

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

part_of

GO:0098799

outer mitochondrial membrane protein complex

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

C

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0090258

negative regulation of mitochondrial fission

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

P

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0070584

mitochondrion morphogenesis

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

P

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0061003

positive regulation of dendritic spine morphogenesis

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

P

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0051646

mitochondrion localization

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0048312

intracellular distribution of mitochondria

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

P

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0046039

GTP metabolic process

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

enables

GO:0042803

protein homodimerization activity

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

F

Seeded From UniProt

complete

part_of

GO:0031307

integral component of mitochondrial outer membrane

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

C

Seeded From UniProt

complete

part_of

GO:0016021

integral component of membrane

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

C

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0010636

positive regulation of mitochondrial fusion

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

P

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0008053

mitochondrial fusion

PMID:12527753[4]

ECO:0000315

mutant phenotype evidence used in manual assertion

MGI:MGI:2450304

P

Seeded From UniProt

complete

acts_upstream_of_or_within

GO:0008053

mitochondrial fusion

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

P

Seeded From UniProt

complete

part_of

GO:0005743

mitochondrial inner membrane

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

C

Seeded From UniProt

complete

part_of

GO:0005741

mitochondrial outer membrane

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

RGD:621460

C

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

PMID:18614015[5]

ECO:0007005

high throughput direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

C

Seeded From UniProt

complete

enables

GO:0003924

GTPase activity

GO_REF:0000096

ECO:0000266

sequence orthology evidence used in manual assertion

UniProtKB:Q8IWA4

F

Seeded From UniProt

complete

involved_in

GO:1990613

mitochondrial membrane fusion

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

P

Seeded From UniProt

complete

part_of

GO:0098799

outer mitochondrial membrane protein complex

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

C

Seeded From UniProt

complete

involved_in

GO:0051646

mitochondrion localization

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

P

Seeded From UniProt

complete

involved_in

GO:0046039

GTP metabolic process

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

P

Seeded From UniProt

complete

part_of

GO:0031307

integral component of mitochondrial outer membrane

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

C

Seeded From UniProt

complete

part_of

GO:0016021

integral component of membrane

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

C

Seeded From UniProt

complete

involved_in

GO:0008053

mitochondrial fusion

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

P

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

C

Seeded From UniProt

complete

enables

GO:0003924

GTPase activity

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q8IWA4
ensembl:ENSP00000420617

F

Seeded From UniProt

complete

enables

GO:0003924

GTPase activity

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR006884
InterPro:IPR027088
InterPro:IPR027094

F

Seeded From UniProt

complete

enables

GO:0005525

GTP binding

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR030381

F

Seeded From UniProt

complete

part_of

GO:0005741

mitochondrial outer membrane

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR006884
InterPro:IPR027088

C

Seeded From UniProt

complete

involved_in

GO:0008053

mitochondrial fusion

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR006884
InterPro:IPR027088

P

Seeded From UniProt

complete

part_of

GO:0016021

integral component of membrane

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR006884
InterPro:IPR027088

C

Seeded From UniProt

complete

involved_in

GO:0007275

multicellular organism development

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0217

P

Seeded From UniProt

complete

enables

GO:0000166

nucleotide binding

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0547

F

Seeded From UniProt

complete

enables

GO:0016787

hydrolase activity

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0378

F

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0496

C

Seeded From UniProt

complete

part_of

GO:0005741

mitochondrial outer membrane

GO_REF:0000037
GO_REF:0000039

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-1000
UniProtKB-SubCell:SL-0172

C

Seeded From UniProt

complete

enables

GO:0005525

GTP binding

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0342

F

Seeded From UniProt

complete

part_of

GO:0016021

integral component of membrane

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0812

C

Seeded From UniProt

complete

part_of

GO:0016020

membrane

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0472

C

Seeded From UniProt

complete

Notes

References

See Help:References for how to manage references in GONUTS.

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Chen, H et al. (2005) Disruption of fusion results in mitochondrial heterogeneity and dysfunction. J. Biol. Chem. 280 26185-92 PubMed GONUTS page
  2. Yue, W et al. (2014) A small natural molecule promotes mitochondrial fusion through inhibition of the deubiquitinase USP30. Cell Res. 24 482-96 PubMed GONUTS page
  3. 3.0 3.1 3.2 3.3 Gaudet, P et al. (2011) Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Brief. Bioinformatics 12 449-62 PubMed GONUTS page
  4. Chen, H et al. (2003) Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. J. Cell Biol. 160 189-200 PubMed GONUTS page
  5. Pagliarini, DJ et al. (2008) A mitochondrial protein compendium elucidates complex I disease biology. Cell 134 112-23 PubMed GONUTS page