Tissue Iron in Friedreich Ataxia
This report summarizes the histology and histopathology of iron in the main tissues affected by FA. None of the affected anatomical sites reveals an elevation of total iron levels. In the myocardium, a small percentage of fibers shows iron-reactive granular inclusions. The accumulation of larger iron aggregates and fiber invasion cause necrosis and damage to the contractile apparatus. In the dentate nucleus, the principal FA-caused tissue injury is neuronal atrophy and grumose reaction. X-ray fluorescence mapping of iron in the dentate nucleus in FA shows retention of the metal in the center of the collapsed structure. Imm...
Source: Journal of Integrative Neuroscience - January 30, 2024 Category: Neuroscience Authors: Arnulf H Koeppen Source Type: research
Tissue Iron in Friedreich Ataxia
This report summarizes the histology and histopathology of iron in the main tissues affected by FA. None of the affected anatomical sites reveals an elevation of total iron levels. In the myocardium, a small percentage of fibers shows iron-reactive granular inclusions. The accumulation of larger iron aggregates and fiber invasion cause necrosis and damage to the contractile apparatus. In the dentate nucleus, the principal FA-caused tissue injury is neuronal atrophy and grumose reaction. X-ray fluorescence mapping of iron in the dentate nucleus in FA shows retention of the metal in the center of the collapsed structure. Imm...
Source: Journal of Integrative Neuroscience - January 30, 2024 Category: Neuroscience Authors: Arnulf H Koeppen Source Type: research
Tissue Iron in Friedreich Ataxia
This report summarizes the histology and histopathology of iron in the main tissues affected by FA. None of the affected anatomical sites reveals an elevation of total iron levels. In the myocardium, a small percentage of fibers shows iron-reactive granular inclusions. The accumulation of larger iron aggregates and fiber invasion cause necrosis and damage to the contractile apparatus. In the dentate nucleus, the principal FA-caused tissue injury is neuronal atrophy and grumose reaction. X-ray fluorescence mapping of iron in the dentate nucleus in FA shows retention of the metal in the center of the collapsed structure. Imm...
Source: Journal of Integrative Neuroscience - January 30, 2024 Category: Neuroscience Authors: Arnulf H Koeppen Source Type: research
Tissue Iron in Friedreich Ataxia
This report summarizes the histology and histopathology of iron in the main tissues affected by FA. None of the affected anatomical sites reveals an elevation of total iron levels. In the myocardium, a small percentage of fibers shows iron-reactive granular inclusions. The accumulation of larger iron aggregates and fiber invasion cause necrosis and damage to the contractile apparatus. In the dentate nucleus, the principal FA-caused tissue injury is neuronal atrophy and grumose reaction. X-ray fluorescence mapping of iron in the dentate nucleus in FA shows retention of the metal in the center of the collapsed structure. Imm...
Source: Journal of Integrative Neuroscience - January 30, 2024 Category: Neuroscience Authors: Arnulf H Koeppen Source Type: research
Correction: Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain
Cell Death & Disease, Published online: 29 January 2024; doi:10.1038/s41419-024-06459-2Correction: Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain (Source: Cell death and disease)
Source: Cell death and disease - January 29, 2024 Category: Internal Medicine Authors: Davide Doni Federica Cavion Marco Bortolus Elisa Baschiera Silvia Muccioli Giulia Tombesi Federica d ’Ettorre Daniele Ottaviani Elena Marchesan Luigi Leanza Elisa Greggio Elena Ziviani Antonella Russo Milena Bellin Geppo Sartori Donatella Carbonera Leon Source Type: research
Omaveloxolone for the treatment of Friedreich ataxia: clinical trial results and practical considerations
Expert Rev Neurother. 2024 Jan 25:1-8. doi: 10.1080/14737175.2024.2310617. Online ahead of print.ABSTRACTINTRODUCTION: Omavaloxolone, an NRF2 activator, recently became the first drug approved specifically for the treatment of Friedreich ataxia (FRDA). This landmark achievement provides a background for a review of the detailed data leading to the approval.AREAS COVERED: The authors review the data from the 4 major articles on FRDA in the context of the authors' considerable (>1000 patients) experience in treating individuals with FRDA. The data is presented in the context not only of its scientific meaning but also in ...
Source: Expert Review of Neurotherapeutics - January 25, 2024 Category: Neurology Authors: David R Lynch Susan Perlman Kim Schadt Source Type: research
Omaveloxolone: a groundbreaking milestone as the first FDA-approved drug for Friedreich ataxia
Trends Mol Med. 2024 Feb;30(2):117-125. doi: 10.1016/j.molmed.2023.12.002. Epub 2024 Jan 24.ABSTRACTFriedreich ataxia (FA) is an inherited autosomal recessive neurodegenerative disease (NDD) characterized primarily by progressive sensory and spinocerebellar ataxia associated with hypertrophic cardiomyopathy. FA is due to an intronic GAA repeat expansion within the frataxin gene (FXN) leading to reduced levels of frataxin (FXN) which causes mitochondrial dysfunction, production of reactive oxygen species (ROS), and altered iron metabolism. To date there is no resolutive cure for FA; however, the FDA has recently approved om...
Source: Trends in Molecular Medicine - January 25, 2024 Category: Molecular Biology Authors: Federica Pilotto Deepika M Chellapandi H élène Puccio Source Type: research
Omaveloxolone for the treatment of Friedreich ataxia: clinical trial results and practical considerations
Expert Rev Neurother. 2024 Jan 25:1-8. doi: 10.1080/14737175.2024.2310617. Online ahead of print.ABSTRACTINTRODUCTION: Omavaloxolone, an NRF2 activator, recently became the first drug approved specifically for the treatment of Friedreich ataxia (FRDA). This landmark achievement provides a background for a review of the detailed data leading to the approval.AREAS COVERED: The authors review the data from the 4 major articles on FRDA in the context of the authors' considerable (>1000 patients) experience in treating individuals with FRDA. The data is presented in the context not only of its scientific meaning but also in ...
Source: Expert Review of Neurotherapeutics - January 25, 2024 Category: Neurology Authors: David R Lynch Susan Perlman Kim Schadt Source Type: research
