FCJ1 was first identified and characterized in yeast, where it was found to be specifically enriched in CJs . The absence of FCJ1 results in the loss of CJs, leading to concentric stacks of inner membrane within the mitochondrial matrix and increased levels of F1F0-ATP synthase supercomplexes . Overexpression of FCJ1 leads to an increase in CJ formation, branching of cristae, enlargement of CJ diameter, and reduced levels of F1F0 supercomplexes .
FCJ1 is part of a large multisubunit complex known as MICOS/MINOS/MitOS, which plays a central role in the formation of CJs and in determining cristae morphology . The C-terminal domain of Fcj1 is crucial for the formation of stable CJs and is required for the genetic interaction of Fcj1 with the F1F0 ATP synthase .
FCJ1 modulates CJ formation in an antagonistic manner to the subunits e and g of the F1F0 ATP synthase, influencing the oligomerization state of the F1F0 ATP synthase, which is essential for cristae structure .
FCJ1 plays a direct role in determining the number and architecture of CJs . Overexpression of FCJ1 increases the number of CJs per cell and leads to increased branching of cristae . Down-regulation of FCJ1 leads to a progressive decrease in the number of CJs and cristae branches .
FCJ1 interacts with the nucleoid protein Abf2, suggesting a role in maintaining the distribution and size of mtDNA nucleoids .
FCJ1 has a regulatory influence on the oligomeric state of F1F0 . Deletion of FCJ1 leads to an increase in the level of F1F0 supercomplexes, while overexpression of FCJ1 leads to a reduction in the level of F1F0 supercomplexes .
A genetic interaction exists between FCJ1 and Su e/Su g of F1F0, placing all proteins in the same pathway . The latter subunits promote the assembly of F1F0-ATP synthase oligomers, whereas FCJ1 has the opposite effect .
The mammalian protein mitofilin/IMMT is likely an orthologue of FCJ1, based on its depletion phenotype .
Recombinant FCJ1 can be produced in vitro using an E. coli expression system .
Recombinant Pichia pastoris Formation of crista junctions protein 1 (FCJ1) is a component of the MICOS complex, a large protein complex within the mitochondrial inner membrane. This complex plays critical roles in maintaining crista junctions, preserving inner membrane architecture, and establishing contact sites with the outer membrane. FCJ1 contributes to the structural integrity of cristae membranes by connecting them to the inner boundary membrane. Furthermore, it facilitates protein import via the mitochondrial intermembrane space assembly (MIA) pathway.
KEGG: ppa:PAS_chr1-4_0172
STRING: 644223.XP_002490286.1