MFN1

The protein encoded by this gene is a mediator of mitochondrial fusion. This protein and mitofusin 2 are homologs of the Drosophila protein fuzzy onion (Fzo). They are mitochondrial membrane proteins that interact with each other to facilitate mitochondrial targeting. [provided by RefSeq, Jul 2008]

mitofusin 1

Mitochondrial outer membrane GTPase that mediates mitochondrial clustering and fusion (PubMed:12475957, PubMed:12759376, PubMed:27920125, PubMed:28114303).

Membrane clustering requires GTPase activity (PubMed:27920125).

It may involve a major rearrangement of the coiled coil domains (PubMed:27920125, PubMed:28114303).

Mitochondria are highly dynamic organelles, and their morphology is determined by the equilibrium between mitochondrial fusion and fission events (PubMed:12475957, PubMed:12759376).

Overexpression induces the formation of mitochondrial networks (in vitro) (PubMed:12759376).

Has low GTPase activity (PubMed:27920125, PubMed:28114303).

GTP metabolic process Source: UniProtKB
Mitochondrial fusion Source: BHF-UCL
Mitochondrial membrane fusion Source: UniProtKB
Mitochondrion localization Source: UniProtKB
Positive regulation of mitochondrial membrane potential Source: Ensembl

Mitochondrion outer membrane
Isoform 2:
Cytoplasm

Cytoplasmic: 1-584
Helical: 585-605
Mitochondrial intermembrane: 606-608
Helical: 609-629
Cytoplasmic: 630-741

Ubiquitinated by non-degradative ubiquitin by PRKN (PubMed:23933751). Deubiquitination by USP30 inhibits mitochondrial fusion (By similarity). Ubiquitinated by MARCHF5 (PubMed:20103533). 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 (PubMed:23933751).