Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

Oleksandr Gakh, Sungjo Park, Gang Liu, +4

Human Molecular Genetics

Reductive StressMitochondrial BiogenesisMitochondrial FunctionBiochemistryLongevity +13

Yeast Frataxin Sequentially Chaperones and Stores Iron by Coupling Protein Assembly with Iron Oxidation

Sungjo Park, Oleksandr Gakh, Heather A. O’Neill, +4

Journal of Biological Chemistry

Physical Evidence that Yeast Frataxin Is an Iron Storage Protein

Oleksandr Gakh, Jiří Adamec, A. Marquis Gacy, +3

Biochemistry

Protein ChemistryProtein SubunitsBiochemistryProtein AssemblyProtein Folding +12

Cleavage Site Selection within a Folded Substrate by the ATP-dependent Lon Protease

Gabriela Ondrovičová, Tong Liu, Kamalendra Singh, +8

Journal of Biological Chemistry

Atp-dependent Lon ProteaseMitochondrial SubstratesProtein AssemblyMolecular BiologyProtein Refolding +16

Two-step Processing of Human Frataxin by Mitochondrial Processing Peptidase

Patrizia Cavadini, Jiří Adamec, Franco Taroni, +2

Journal of Biological Chemistry

Assembly of Human Frataxin Is a Mechanism for Detoxifying Redox-Active Iron

Heather A. O’Neill, Oleksandr Gakh, Sungjo Park, +5

Biochemistry

Molecular BiologyChemical BiologyHuman FrataxinRedox BiologyOxidative Stress +17

The Ferroxidase Activity of Yeast Frataxin

Sungjo Park, Oleksandr Gakh, Steven M. Mooney, +1

Journal of Biological Chemistry

The Structures of Frataxin Oligomers Reveal the Mechanism for the Delivery and Detoxification of Iron

T. Karlberg, Ulrika Schagerlöf, Oleksandr Gakh, +7

Structure

BiochemistryIron MetabolismNatural SciencesMolecular BiologyFrataxin Oligomers Reveal +5

<i>PMPCA</i>mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia

Rebekah Jobling, Mirna Assoum, Oleksandr Gakh, +20

Brain

Normal and Friedreich Ataxia Cells Express Different Isoforms of Frataxin with Complementary Roles in Iron-Sulfur Cluster Assembly

Oleksandr Gakh, Tibor Bedekovics, Samantha F. Duncan, +3

Journal of Biological Chemistry