Cellular and functional analysis of four mutations located in the mitochondrial ATPase6 gene.

Abstract

The smallest rotary motor of living cells, F0F1-ATP synthase, couples proton flow-generated by the OXPHOS system-from the intermembrane space back to the matrix with the conversion of ADP to ATP. While all mutations affecting the multisubunit complexes of the OXPHOS system probably impact on the cell's output of ATP, only mutations in complex V can be… (More)
DOI: 10.1002/jcb.22055

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