|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
In addition, different models of cardiac iron dysmetabolism will be discussed through the examples of heart failure (cardiomyocyte iron deficiency), myocardial infarction (acute changes in cardiac iron turnover), doxorubicin-induced cardiotoxicity (cardiomyocyte iron overload in mitochondria), thalassaemia (cardiomyocyte cytosolic and mitochondrial iron overload) and Friedreich ataxia (asymmetric cytosolic/mitochondrial cardiac iron dysmetabolism).
|
31162883 |
2020 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
Mechanisms of mitochondrial iron overload are discussed considering the potential roles of frataxin in the major mitochondrial metabolic pathways that use iron.
|
30833885 |
2019 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
Mitochondria iron overload is a key feature of the neurodegenerative disease Friedreich's ataxia (FRDA).
|
30778428 |
2019 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
These results suggest that TPP-DFO could be an interesting alternative for the treatment of mitochondrial iron overload e.g. in Friedreich's ataxia.
|
28770399 |
2017 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
Silencing of FXN expressions occurred both in somatic FRDA-skin fibroblasts and two of the induced pluripotent stem cells (iPSC) clones; a sign of stress condition was shown in FRDA-iPSC cardiomyocytes with disorganized mitochondrial network and mitochondrial DNA (mtDNA) depletion; hypertrophic cardiac stress responses were observed by an increase in α-actinin-positive cell sizes revealed by FACS analysis as well as elevation in brain natriuretic peptide (BNP) gene expression; the intracellular iron accumulated in FRDA cardiomyocytes might be due to attenuated negative feedback response of transferring receptor (TSFR) expression and positive feedback response of ferritin (FTH1); energy synthesis dynamics, in terms of ATP production rate, was impaired in FRDA-iPSC cardiomyocytes, which were prone to iron overload condition.
|
24327207 |
2014 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
The physiopathological consequences of frataxin deficiency are a severe disruption of iron-sulfur cluster biosynthesis, mitochondrial iron overload coupled to cellular iron dysregulation and an increased sensitivity to oxidative stress.
|
20413654 |
2010 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
Current evidence suggests that loss of frataxin causes iron overload in tissues, and increase in free-radical production leading to oxidation and inactivation of mitochondrial respiratory chain enzymes, particularly Complexes I, II, III and aconitase.
|
19748629 |
2009 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
The mitochondrial iron overload and presumed cytosolic iron depletion potentially further compromise function in frataxin-deficient cells by decreasing frataxin expression.
|
18424449 |
2008 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
Mitochondria iron overload occurs also in cells deficient in frataxin, a mitochondrial protein involved in iron handling and implicated in Friedreich ataxia.
|
15282205 |
2004 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
It is, however, still unclear whether frataxin is directly involved in iron binding, since the yeast orthologue, but not the human protein, has been shown to form large aggregates in the presence of large iron excess.
|
12140189 |
2002 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
Many of these disorders are characterized by iron overload and include relatively common diseases such as hereditary hemochromatosis, rare abnormalities of plasma protein synthesis (atransferrinemia and aceruloplasminemia), and the neuromuscular disease Friedreich ataxia.
|
11419690 |
2001 |
|
Iron Overload
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
This chapter considers the most common inherited disorder in individuals of European ancestry (hereditary hemochromatosis), a widespread disease in sub-Saharan populations for which the genetic basis is still uncertain (African dietary iron overload), and several less frequent or rare disorders (juvenile hemochromatosis, atransferrinemia, aceruloplasminemia, hyperferritinemia with autosomal dominant congenital cataract, Friedreich's ataxia, and X-linked sideroblastic anemia with ataxia).
|
10774476 |
2000 |
|
Iron Overload
|
0.100 |
Biomarker
|
disease |
BEFREE |
In Saccharomyces cerevisiae, lack of the yeast frataxin homolog ( YFH1 gene, Yfh1p polypeptide) results in mitochondrial iron accumulation, suggesting that frataxin is required for mitochondrial iron homeostasis and that FRDA results from oxidative damage secondary to mitochondrial iron overload.
|
10332043 |
1999 |