Results 201 to 210 of about 10,061 (229)

Robust behavioral assessment of the inducible Friedreich's ataxia mouse does not show improvement with NRF2 induction.

Dis Model Mech
Montgomery CB, Salinas L, Cox GP, Adcock LE, Chang T, Figueroa F, Cortopassi G, Dedkova EN.   +7 more
europepmc   +1 more source

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Frataxin inhibits the sensitivity of the myocardium to ferroptosis by regulating iron homeostasis.

Free Radical Biology & Medicine, 2023
RATIONALE Myocardial ischemia/reperfusion (I/R) injury is characterized by cell death via various cellular mechanisms upon reperfusion. As a new type of cell death, ferroptosis provides new opportunities to reduce myocardial cell death.
Zihui Zhang, Wenhua Jiang, Chang-xiong Zhang, Yue Yin, N. Mu, Yishi Wang, Lu Yu, Heng Ma   +7 more
semanticscholar   +1 more source

Drosophila melanogaster frataxin: protein crystal and predicted solution structure with identification of the iron-binding regions.

Acta Crystallographica Section D: Structural Biology, 2023
Friedreich's ataxia (FRDA) is a hereditary cardiodegenerative and neurodegenerative disease that affects 1 in 50 000 Americans. FRDA arises from either a cellular inability to produce sufficient quantities or the production of a nonfunctional form of the
A. Rodrigues, Sharon Batelu, Tiara V. Hinton, J. Rotondo, Lindsey Thompson, J. Brunzelle, Timothy L. Stemmler   +6 more
semanticscholar   +1 more source

Frataxin fracas

Nature Genetics, 1997
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Cossee M, Campuzano V, Koutnikova H, Fischbeck K, Mandel JL, Koenig M, Bidichandani SI, Patel PI, Molte MD, Canizares J, DeFrutos R, Pianese L, Cavalcanti F, MONTICELLI A, Cocozza S, Montermini L, Pandolfo M   +16 more
openaire   +3 more sources

Iron-frataxin involved in the protective effect of quercetin against alcohol-induced liver mitochondrial dysfunction.

Journal of Nutritional Biochemistry, 2022
Emerging evidence supports the beneficial effect of quercetin on liver mitochondrial disorders. However, the molecular mechanism by which quercetin protects mitochondria is limited, especially in alcoholic liver disease. In this study, C57BL/6N mice were
Jing-jing Liu, Huimin Chen, Hongkun Lin, Shufen Peng, Li Chen, Xue-Er Cheng, Ping Yao, Yuhan Tang   +7 more
semanticscholar   +1 more source

Functional studies of frataxin

Acta Paediatrica, 2004
Mitochondria generate adenosine triphosphate (ATP) but also dangerous reactive oxygen species (ROS). One‐electron reduction of dioxygen in the early stages of the electron transport chain yields a superoxide radical that is detoxified by mitochondrial superoxide dismutase to give hydrogen peroxide.
G, Isaya, H A, O'Neill, O, Gakh, S, Park, R, Mantcheva, S M, Mooney   +5 more
openaire   +2 more sources

Glial overexpression of Tspo extends lifespan and protects against frataxin deficiency in Drosophila.

Biochimie
The translocator protein TSPO is an evolutionary conserved mitochondrial protein overexpressed in various contexts of neurodegeneration. Friedreich Ataxia (FA) is a neurodegenerative disease due to GAA expansions in the FXN gene leading to decreased ...
Estelle Jullian, M. Russi, Ema Turki, Margaux Bouvelot, Laura Tixier, Sandrine Middendorp, Elodie Martin, Véronique Monnier   +7 more
semanticscholar   +1 more source

Frataxin and frataxin deficiency in Friedreich's ataxia

Journal of the Neurological Sciences, 2003
A deficiency in the protein frataxin causes Friedreich’s ataxia (FRDA). Missense mutations in f2% of disease alleles cause a functional deficiency of frataxin, while f98% of disease alleles have a guanine-adenine-adenine (GAA) repeat expansion in the first intron [1]. The expansion decreases frataxin expression by interfering with transcription [2].
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Frataxin Structure and Function

2019
Mammalian frataxin is a small mitochondrial protein involved in iron sulfur cluster assembly. Frataxin deficiency causes the neurodegenerative disease Friedreich's Ataxia. Valuable knowledge has been gained on the structural dynamics of frataxin, metal-ion-protein interactions, as well as on the effect of mutations on protein conformation, stability ...
Castro I. H., Pignataro M. F., Sewell K. E., Espeche L. D., Herrera M. G., Noguera M. E., Dain L., Nadra A. D., Aran M., Smal C., Gallo M., Santos J.   +11 more
openaire   +4 more sources