Zobrazeno 1 - 10
of 27
pro vyhledávání: '"Fabien, Delaspre"'
Autor:
Arabela Sanz-Alcázar, Marta Portillo-Carrasquer, Fabien Delaspre, Maria Pazos-Gil, Jordi Tamarit, Joaquim Ros, Elisa Cabiscol
Publikováno v:
Redox Biology, Vol 76, Iss , Pp 103339- (2024)
Friedreich ataxia (FA) is a rare neurodegenerative disease caused by decreased levels of the mitochondrial protein frataxin. Frataxin has been related in iron homeostasis, energy metabolism, and oxidative stress. Ferroptosis has recently been shown t
Externí odkaz:
https://doaj.org/article/9b59a3a421f5404c971987defaf49e32
Autor:
Laura Rodríguez-Pascau, Elena Britti, Pablo Calap-Quintana, Yi Na Dong, Cristina Vergara, Fabien Delaspre, Marta Medina-Carbonero, Jordi Tamarit, Federico V. Pallardó, Pilar Gonzalez-Cabo, Joaquim Ros, David R. Lynch, Marc Martinell, Pilar Pizcueta
Publikováno v:
Neurobiology of Disease, Vol 148, Iss , Pp 105162- (2021)
Friedreich ataxia (FRDA), the most common autosomal recessive ataxia, is characterized by degeneration of the large sensory neurons and spinocerebellar tracts, cardiomyopathy, and increased incidence in diabetes. The underlying pathophysiological mec
Externí odkaz:
https://doaj.org/article/d8901ebb28414e638a9b30b6d425544b
Autor:
Arabela Sanz-Alcázar, Elena Britti, Fabien Delaspre, Marta Medina-Carbonero, Maria Pazos-Gil, Jordi Tamarit, Joaquim Ros, Elisa Cabiscol
Friedreich ataxia (FA) is a rare, recessive neuro-cardiodegenerative disease caused by deficiency of the mitochondrial protein frataxin. Mitochondrial dysfunction, a reduction in the activity of iron-sulfur enzymes, iron accumulation, and increased o
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::11690086071ba22b0e8bd95fbb1cadd7
https://doi.org/10.1101/2023.02.01.526688
https://doi.org/10.1101/2023.02.01.526688
Autor:
Rui, Alves, Maria, Pazos-Gil, Marta, Medina-Carbonero, Arabela, Sanz-Alcázar, Fabien, Delaspre, Jordi, Tamarit
Publikováno v:
International journal of molecular sciences. 23(21)
Friedreich's ataxia is a neurodegenerative disease caused by mutations in the frataxin gene. Frataxin homologues, including bacterial CyaY proteins, can be found in most species and play a fundamental role in mitochondrial iron homeostasis, either pr
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::3212a204785e098a7028b8841018bfb8
https://pubmed.ncbi.nlm.nih.gov/36361939
https://pubmed.ncbi.nlm.nih.gov/36361939
Publikováno v:
Neurochemical Research. 46:108-119
Calpains are calcium-dependent proteases activated in apoptotic cell death and neurodegeneration. Friedreich Ataxia is a neurodegenerative rare disease caused by frataxin deficiency, a mitochondrial protein. Dorsal root ganglion (DRG) sensory neurons
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::f57573929d3ce196f1ee592d0b4788ab
https://doi.org/10.1007/s11064-020-03020-3
https://doi.org/10.1007/s11064-020-03020-3
Autor:
Rui Alves, Maria Pazos-Gil, Marta Medina-Carbonero, Arabela Sanz-Alcázar, Fabien Delaspre, Jordi Tamarit
Publikováno v:
International Journal of Molecular Sciences; Volume 23; Issue 21; Pages: 13151
Friedreich’s ataxia is a neurodegenerative disease caused by mutations in the frataxin gene. Frataxin homologues, including bacterial CyaY proteins, can be found in most species and play a fundamental role in mitochondrial iron homeostasis, either
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ed72e0e7262c5aa3697d7d69aeea2c0f
https://hdl.handle.net/10459.1/84485
https://hdl.handle.net/10459.1/84485
Autor:
Marta Medina-Carbonero, Arabela Sanz-Alcázar, Elena Britti, Fabien Delaspre, Elisa Cabiscol, Joaquim Ros, Jordi Tamarit
Publikováno v:
Cellular and Molecular Life Sciences
Repositorio Abierto de la UdL
Universitad de Lleida
Repositorio Abierto de la UdL
Universitad de Lleida
Friedreich Ataxia (FA) is a rare neuro-cardiodegenerative disease caused by mutations in the frataxin (FXN) gene. The most prevalent mutation is a GAA expansion in the first intron of the gene causing decreased frataxin expression. Some patients pres
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a27b8288178e058258981a14cf978b28
http://europepmc.org/articles/PMC8763788
http://europepmc.org/articles/PMC8763788
Autor:
Guangliang Wang, Surendra K Rajpurohit, Fabien Delaspre, Steven L Walker, David T White, Alexis Ceasrine, Rejji Kuruvilla, Ruo-jing Li, Joong S Shim, Jun O Liu, Michael J Parsons, Jeff S Mumm
Publikováno v:
eLife, Vol 4 (2015)
Whole-organism chemical screening can circumvent bottlenecks that impede drug discovery. However, in vivo screens have not attained throughput capacities possible with in vitro assays. We therefore developed a method enabling in vivo high-throughput
Externí odkaz:
https://doaj.org/article/6bc5a75f96814fc3aa7ccaecb3f5d1b1
Biochemical alterations precede neurobehavioral deficits in a novel mouse model of Friedreich ataxia
Autor:
Jordi Tamarit, Elisa Cabiscol, Fabien Delaspre, Joaquim Ros, A. Sanz-Alcázar, Elena Britti, Marta Medina-Carbonero
Friedreich Ataxia (FA) is a rare neuro-cardiodegenerative disease, caused by partial deficiency of frataxin, a mitochondrial protein. This deficiency is caused by the presence of a GAA triplet expansion in the first intron of the frataxin gene or, in
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::87c33d7c95cefa57ac0babc0f5ef0a87
https://doi.org/10.1101/2021.04.05.438486
https://doi.org/10.1101/2021.04.05.438486
Autor:
Arabela Sanz-Alcázar, Joaquim Ros, Fabien Delaspre, Jordi Tamarit, Elisa Cabiscol, Elena Britti, Marta Medina-Carbonero
Publikováno v:
IUBMB lifeREFERENCES. 73(3)
Friedreich Ataxia is a neuro-cardiodegenerative disease caused by the deficiency of frataxin, a mitochondrial protein. Many evidences indicate that frataxin deficiency causes an unbalance of iron homeostasis. Nevertheless, in the last decade many res
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::02b2971620dfdc69404a6afd0229c6af
https://pubmed.ncbi.nlm.nih.gov/33675183
https://pubmed.ncbi.nlm.nih.gov/33675183