Mitofusin 1

Expressed in 8.5 dpc, 9.5 dpc, 10.5 dpc and 11.5 dpc embryos.

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.

Mitochondrial outer membrane GTPase that mediates mitochondrial clustering and fusion (PubMed:12527753, PubMed:15297672, PubMed:23921378, PubMed:24513856). Membrane clustering requires GTPase activity (By similarity). It may involve a major rearrangement of the coiled coil domains (PubMed:15297672). Mitochondria are highly dynamic organelles, and their morphology is determined by the equilibrium between mitochondrial fusion and fission events (PubMed:12527753). Overexpression induces the formation of mitochondrial networks (in vitro). Has low GTPase activity (By similarity).

Ubiquitinated by MARCHF5 (By similarity). 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. Ubiquitinated by non-degradative ubiquitin by PRKN, promoting mitochondrial fusion; deubiquitination by USP30 inhibits mitochondrial fusion (PubMed:24513856).

Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. Mitofusin subfamily.

Detected in adult heart (PubMed:12759376). Detected in embryos (at protein level) (PubMed:12527753). Widely expressed (PubMed:11950885).

• Mitochondrion outer membrane
• Multi-pass membrane protein

Mitofusin-1, Transmembrane GTPase MFN1, Mfn1

• entrezGene:67414
• swissprot:Q811U4

Proteins

Immunology & Infectious Disease

83726Da