A5067488862- Mitochondrial Function and Pathology
- Genetic Neurodegenerative Diseases
- Autophagy in Disease and Therapy
- Endoplasmic Reticulum Stress and Disease
- Amyotrophic Lateral Sclerosis Research
- Photosynthetic Processes and Mechanisms
- Sirtuins and Resveratrol in Medicine
- Ferroptosis and cancer prognosis
- Neurological and metabolic disorders
Biomedical Research Institute of Lleida
2022-2025
Universitat de Lleida
2022-2025
Friedreich ataxia (FA) is a rare, recessive neuro-cardiodegenerative disease caused by deficiency of the mitochondrial protein frataxin. Mitochondrial dysfunction, reduction in activity iron-sulfur enzymes, iron accumulation, and increased oxidative stress have been described. Dorsal root ganglion (DRG) sensory neurons are among cellular types most affected early stages this disease. However, its effect on function remains to be elucidated. In present study, we found that primary cultures...
Friedreich ataxia (FA) is a rare, recessive neuro-cardiodegenerative disease caused by deficiency of the mitochondrial protein frataxin. Mitochondrial dysfunction, reduction in activity iron-sulfur enzymes, iron accumulation, and increased oxidative stress have been described. However, mechanisms causing such cellular disturbances mammals are not completely understood. Dorsal root ganglion (DRG) sensory neurons among types most affected early stages this disease. We previously demonstrated...
ABSTRACT 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 recently shown to be involved FA cellular degeneration; however, its role dorsal root ganglion (DRG) sensory neurons, cells that are affected most earliest, mostly unknown. In this study, we used primary cultures frataxin-deficient DRG neurons as well from FXN...
ABSTRACT Friedreich Ataxia (FA) is a cardio-neurodegenerative disease caused by mutations in the frataxin gene, which result low expression. It well-established that deficiency affects iron homeostasis, but tissue-specificity of these alterations poorly understood. In this study, we have analyzed homeostasis FXNI151F mouse model, presents systemic and neurological defects resembling FA patients. Iron overload observed brain from 21-week old mice, both males females, it does not further...
Friedreich's ataxia is a neurodegenerative disease caused by mutations in the frataxin gene. Frataxin homologues, including bacterial CyaY proteins, can be found most species and play fundamental role mitochondrial iron homeostasis, either promoting assembly into metaloproteins or contributing to detoxification. While several lines of evidence suggest that eukaryotic frataxins are more effective than ones detoxification, residues involved this gain function unknown. In work, we analyze...
ABSTRACT Friedreich ataxia (FA) is a rare, recessive neuro-cardiodegenerative disease caused by deficiency of the mitochondrial protein frataxin. Mitochondrial dysfunction, reduction in activity iron-sulfur enzymes, iron accumulation, and increased oxidative stress have been described. However, mechanisms causing such cellular disturbances mammals are not completely understood. Dorsal root ganglion (DRG) sensory neurons among types most affected early stages this disease. We previously...