Results 151 to 160 of about 3,918 (176)

Mechanisms of iron and copper–frataxin interactions

Metallomics, 2017
Investigation of the mechanisms of mitochondrial metal binding to frataxinin vitro.
T. H. L. Han, J. M. Camadro, R. Santos, E. Lesuisse, J. M. El Hage Chahine, N. T. Ha-Duong   +5 more
openaire   +3 more sources

Correlation of frataxin content in blood and skeletal muscle endorses frataxin as a biomarker in Friedreich ataxia

Movement Disorders, 2011
AbstractBackground:Friedreich ataxia is an autosomal recessive disorder caused by mutations in the frataxin gene, leading to reduced levels of the mitochondrial protein frataxin. Assays to quantitatively measure frataxin in peripheral blood have been established.
Wolfgang, Nachbauer, Julia, Wanschitz, Hannes, Steinkellner, Andreas, Eigentler, Brigitte, Sturm, Kurt, Hufler, Barbara, Scheiber-Mojdehkar, Werner, Poewe, Markus, Reindl, Sylvia, Boesch   +9 more
openaire   +2 more sources

Candida albicans lacking the frataxin homologue: a relevant yeast model for studying the role of frataxin

Molecular Microbiology, 2004
SummaryWe cloned the CaYFH1 gene that encodes the yeast frataxin homologue in Candida albicans. CaYFH1 was expressed in Δyfh1 Saccharomyces cerevisiae cells, where it compensated for all the phenotypes tested except for the lack of cytochromes. Double ΔCayfh1/ΔCayfh1 mutant had severe defective growth, accumulated iron in their mitochondria, lacked ...
Renata, Santos, Nicole, Buisson, Simon A B, Knight, Andrew, Dancis, Jean-Michel, Camadro, Emmanuel, Lesuisse   +5 more
openaire   +2 more sources

Iron metabolism in mice with partial frataxin deficiency

The Cerebellum, 2003
Friedreich ataxia (FRDA), the most common autosomal recessive inherited ataxic disorder, is the consequence of deficiency of the mitochondrial protein frataxin, typically caused by homozygous intronic GAA expansions in the corresponding gene. The yeast frataxin homologue (yfh1p) is required for cellular respiration.
Santos, M M, Miranda, C, Levy, Joanne E, Montross, Lynne K, Cossée, Mireille, Sequeiros, Jorge, Andrews, Nancy, Koenig, Michel, Pandolfo, Massimo   +8 more
openaire   +2 more sources

Frataxin: a protein in search for a function

Journal of Neurochemistry, 2013
AbstractReduced levels of the protein frataxin cause the neurodegenerative disease Friedreich's ataxia. Pathology is associated with disruption of iron–sulfur cluster biosynthesis, mitochondrial iron overload, and oxidative stress. Frataxin is a highly conserved iron‐binding protein present in most organisms.
Annalisa Pastore, Helene Puccio
openaire   +2 more sources

The frataxin story

Neuromuscular Disorders, 1997
Victoria Campuzano, Hana Koutnikova, Françoise Foury, Mireille Cossée, Ornella Cazzalini, Michel Koenig   +5 more
openaire   +1 more source

Waiting for frataxin

Nature Genetics, 1997
Joseph V. Gray, Keith J. Johnson
openaire   +1 more source

Oxidative stress-dependent frataxin inhibition mediated alcoholic hepatocytotoxicity through ferroptosis

Toxicology, 2020
Ping Yao, Yuhan Tang
exaly  

Frataxin Deacetylation in Macrophages: Avoiding SIRTain Myocyte Death

Circulation Research, 2023
Ronald J. Vagnozzi, Emma L. Robinson
openaire   +2 more sources

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin

Nature Genetics, 1997
Victoria Campuzano, Pascal Dollé
exaly