Role Of Opa1 Adoa-Causing Mutants In Mitochondrial Nucleoid Distribution

Mitochondria are highly dynamic organelles. They continuously move inside the cell and undergo fusion/fission cycles. Mitochondrial genome (mtDNA) integrity determines mitochondrial function and is packaged into nucleoids by interaction with the mitochondrial transcription factor, TFAM. Previous studies showed that mitochondrial nucleoids undergo dynamic clustering, interact with the inner mitochondrial membrane (IMM), and their abundance and distribution are linked to mitochondrial dynamics. IMM fusion and cristae are mediated by OPA1.