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Proc Natl Acad Sci U S A. 2000 Oct 24;97(22):12239-43.

Frataxin activates mitochondrial energy conversion and oxidative phosphorylation.

Author information

1
Joslin Diabetes Center, Harvard Medical School, Research Division, Boston, MA 02215, USA. michael.ristow@uni-koeln.de

Abstract

Friedreich's ataxia (FA) is an autosomal recessive disease caused by decreased expression of the mitochondrial protein frataxin. The biological function of frataxin is unclear. The homologue of frataxin in yeast, YFH1, is required for cellular respiration and was suggested to regulate mitochondrial iron homeostasis. Patients suffering from FA exhibit decreased ATP production in skeletal muscle. We now demonstrate that overexpression of frataxin in mammalian cells causes a Ca(2+)-induced up-regulation of tricarboxylic acid cycle flux and respiration, which, in turn, leads to an increased mitochondrial membrane potential (delta psi(m)) and results in an elevated cellular ATP content. Thus, frataxin appears to be a key activator of mitochondrial energy conversion and oxidative phosphorylation.

PMID:
11035806
PMCID:
PMC17325
DOI:
10.1073/pnas.220403797
[Indexed for MEDLINE]
Free PMC Article
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