Results 121 to 130 of about 5,449 (210)

Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm.

, 2001
Deficiency of the mitochondrial matrix protein frataxin causes Friedreich ataxia. Frataxin function is believed to be related to mitochondrial iron metabolism and free radical production.
Pandolfo, Massimo, Ohshima, K, Santos, M M   +2 more
core  

Frataxin expression levels.

, 2014
(a) FXN mRNA expression was analyzed by qRT-PCR of Y47R and YG8R fibroblasts, NSCs and differentiated NSCs. The mean values of NSCs and differentiated NSCs data are normalized to the mean FXN mRNA level of the Y47R fibroblasts taken as 100%.
Chiranjeevi Sandi (283956), Vahid Ezzatizadeh (525611), Mark A. Pook (126056), Harvinder Jassal (525610), Sara Anjomani-Virmouni (525612), Madhavi Sandi (525609), Sahar Al-Mahdawi (126054)   +6 more
core   +1 more source

Reply to “Frataxin fracas” [PDF]

Nature Genetics, 1997
Susan Chamberlain, Mark Pook, Jaime Carvajal, Kitm Doudney, Renate Hillermann   +4 more
openaire   +1 more source

Nanobodies as tools for studying human frataxin biology

Communications Biology
Iron-sulfur clusters are essential cofactors for the accurate cellular function of many proteins. In eukaryotic cells, the biogenesis of most iron-sulfur clusters occurs in the mitochondria and involves the action of the Cys desulfurase supercomplex, which is activated by the protein frataxin (FXN).
María Florencia Pignataro, Natalia Brenda Fernández, Alba Garay-Alvarez, María Florencia Pavan, Rafael Molina, Inés G. Muñoz, Julián Grossi, Martín Noguera, Antonella Vila, Augusto E. García, Hernán G. Gentili, Naira Antonia Rodríguez, Martín Aran, Viviana Parreño, Marina Bok, Juan A. Hermoso, Lorena Itatí Ibañez, Javier Santos   +17 more
openaire   +3 more sources

Iron coordination and protein-protein interactions of the protein frataxin

, 2014
Frataxin is a mitochondrial iron metallochaperone that transports ferrous iron to proteins that require it for function. This dissertation research explores the iron binding properties of human frataxin and how frataxin interacts with the mitochondrial ...
Frantom, Patrick, Marcus, Stevan, Gentry-Dye, Leslie, Busenlehner, Laura S., Woski, Stephen, University of Alabama. Dept. of Chemistry, Street, Shane   +6 more
core   +1 more source

Functional characterization of Frataxin isoforms and mechanisms of regulation of frataxin expression

, 2019
Esta tesis tiene embargado el acceso al texto completo hasta el 04-01 ...
openaire   +1 more source

Frataxin and the stress response

, 2010
La frataxina è una proteina mitocondriale, la cui ridotta espressione è responsabile di una malattia neurodegenerativa ereditaria, l’atassia di Friedreich (FRDA).

core  

Mutation in Fe-S scaffold Isu bypasses frataxin deletion.

, 2011
International audienceFrataxin is a conserved mitochondrial protein deficient in patients with Friedreich's ataxia. Frataxin has been implicated in control of iron homeostasis and Fe-S cluster assembly.
Pain, Debkumar, Pain, Jayashree, Yoon, Heeyong, Lesuisse, Emmanuel, Dancis, Andrew, Lyver, Elise R, Golla, Ramesh, Donald, Jason   +7 more
core   +1 more source

The structural characterization of frataxin ion binding sites

, 2006
Frataxin is a protein responsible for the Friedreich ataxia. Frataxin is involved in iron homeostasis and in iron-sulfur assembly. It has been suggested that frataxin acts as an iron chaperone in the monomeric form and as iron storage in the oligomeric ...
MAZZARELLA, LELIO, SICA, FILOMENA, A. Pastore, FRANZESE, MARISA, S. Adinolfi   +4 more
core  

Recent developments in Friedreich's ataxia: a state-of-the-art review. [PDF]

Brain Commun
Chapman LR, Mortiboys H, Shaw PJ.
europepmc   +1 more source