Title : Posttranslational regulation of mitochondrial frataxin and identification of compounds that increase frataxin levels in Friedreich's ataxia.

Pub. Date : 2022 Apr 25

PMID : 35472330






6 Functional Relationships(s)
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1 Friedreich"s Ataxia (FRDA) is a degenerative disease caused by a decrease in the mitochondrial protein frataxin, which is involved in iron-sulfur cluster (ISC) synthesis. Iron frataxin Homo sapiens
2 Friedreich"s Ataxia (FRDA) is a degenerative disease caused by a decrease in the mitochondrial protein frataxin, which is involved in iron-sulfur cluster (ISC) synthesis. Iron frataxin Homo sapiens
3 Diminutions in frataxin result in decreased ISC synthesis, increased mitochondrial iron accumulation, and impaired mitochondrial function. Iron frataxin Homo sapiens
4 We demonstrate that the mitochondrial Lon protease is involved in frataxin degradation and that iron export through the mitochondrial metal transporter Mmt1 protects yeast frataxin from degradation. Iron frataxin Homo sapiens
5 We also determined that when FRDA fibroblasts were grown in media containing elevated iron, mitochondrial ROS increased and frataxin decreased compared to WT fibroblasts. Iron frataxin Homo sapiens
6 We also determined that when FRDA fibroblasts were grown in media containing elevated iron, mitochondrial ROS increased and frataxin decreased compared to WT fibroblasts. Iron frataxin Homo sapiens