Abstract:
:Mutations in the human mtDNA gene encoding subunit III of cytochrome c oxidase (CO) have been reported to cause MELAS and LHON. Poracoccus denitrificans cells expressing substitutions homologous to these MELAS- and LHON-causing mutations had lower growth yield than wild type cells and lower efficiency of proton pumping by CO (e.g. lower H+/e ratio and lower deltapsi), but had similar CO activity. These results indicate that both substitutions (F263L > A212T) cause intrinsic uncoupling, which may be the direct cause of the diseases. These results also suggest that subunit III is involved in proton pumping.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Mather MW,Rottenberg Hdoi
10.1016/s0014-5793(98)00891-6subject
Has Abstractpub_date
1998-08-14 00:00:00pages
93-7issue
1-2eissn
0014-5793issn
1873-3468pii
S0014-5793(98)00891-6journal_volume
433pub_type
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