145525

Sheep Anti-Mitofusin 1 (MFN-1, Hfzo1, Hfzo2, MFN1, Fzo Homolog, Transmembrane GTPase MFN-1)

MFN­1 (Mitofusin 1; also Fzo/"fuzzy onions" homolog and Transmembrane GTPase MFN­1) is an 80­90kD mitochondrial member of the dynamin family of molecules. It is ubiquitously expressed, and found in the outer mitochondrial membrane. MFN­1 apparently mediates the fusion of individual mitochondria with each other through trans­interactions with adjacent mitochondrial MFN­1 and/or MFN­2. Pertubations with this process result in both a general overall fragmentation of mitochondria, and in cell­specific effects such as a reduction in oxidative phosphorylation or an imbalance in the generation of ROS. MFN­1 has two key domains. One is a coiled­coil region that mediates MFN­1:MFN­1/2 binding, and a second is a GTPase domain whose cleavage of GTP is necessary for membrane fusion. Human MFN­1 is a 741 amino acid (aa) two transmembrane protein that contains a cytoplasmic N­ and C­terminus. The cytoplasmic N­terminus (aa1­584) contains the GTPase domain (aa82­286) while the cytoplasmic C­terminus possesses a critical coiled­coil motif (aa679­734). MFN­1 is known to form oligomers, either with itself or MFN­2, and to undergo ubiquitination by MARCH5. Multiple splice variants are reported to occur. One is cytosolic and contains a four aa substitution for aa367­741. Three others show a deletion of aa444­554, aa326­408 and an alternative start site at Met387, respectively. Over aa1­77, human and mouse MFN­1 share 88% aa sequence identity. Full­length human MFN­1 and MFN­2 share 61% aa sequence identity.