Mitochondrial iron and calcium homeostasis in Friedreich ataxia

IUBMB Life ◽  
2021 ◽  
Vol 73 (3) ◽  
pp. 543-553 ◽  
Author(s):  
Jordi Tamarit ◽  
Elena Britti ◽  
Fabien Delaspre ◽  
Marta Medina‐Carbonero ◽  
Arabela Sanz‐Alcázar ◽  
...  
Keyword(s):  
Calcium Homeostasis ◽  
Friedreich Ataxia ◽  
Mitochondrial Iron ◽  
Iron And Calcium

Targeting of the mouse Slc39a2 (Zip2) gene reveals highly cell-specific patterns of expression, and unique functions in zinc, iron, and calcium homeostasis

genesis ◽  
2007 ◽  
Vol 45 (6) ◽  
pp. 339-352 ◽  
Author(s):  
Jennifer L. Peters ◽  
Jodi Dufner-Beattie ◽  
Wenhao Xu ◽  
Jim Geiser ◽  
Brett Lahner ◽  
...  
Keyword(s):  
Calcium Homeostasis ◽  
Iron And Calcium

Direct evidence that mitochondrial iron accumulation occurs in Friedreich ataxia

1999 ◽  
Vol 45 (5) ◽  
pp. 673-675 ◽  
Author(s):  
Martin B. Delatycki ◽  
James Camakaris ◽  
Hilary Brooks ◽  
Tracy Evans-Whipp ◽  
David R. Thorburn ◽  
...  
Keyword(s):  
Direct Evidence ◽  
Friedreich Ataxia ◽  
Iron Accumulation ◽  
Mitochondrial Iron

scholarly journals Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron availability: hypothesis for a protective role in Friedreich ataxia

2008 ◽  
Vol 18 (1) ◽  
pp. 1-11 ◽  
Author(s):  
A. Campanella ◽  
E. Rovelli ◽  
P. Santambrogio ◽  
A. Cozzi ◽  
F. Taroni ◽  
...  
Keyword(s):  
Oxidative Damage ◽  
Friedreich Ataxia ◽  
Protective Role ◽  
Iron Availability ◽  
Mitochondrial Iron

scholarly journals Therapeutic Developments in Friedreich Ataxia

2012 ◽  
Vol 27 (9) ◽  
pp. 1212-1216 ◽  
Author(s):  
Robert B. Wilson
Keyword(s):  
Oxidative Stress ◽  
Matrix Protein ◽  
Disease Gene ◽  
Cluster Formation ◽  
Friedreich Ataxia ◽  
Iron Accumulation ◽  
Iron Sulfur Cluster ◽  
Iron Sulfur Clusters ◽  
Iron Sulfur ◽  
Mitochondrial Iron

Friedreich ataxia is an inherited, severe, progressive neuro- and cardiodegenerative disorder for which there currently is no approved therapy. Friedreich ataxia is caused by the decreased expression and/or function of frataxin, a mitochondrial matrix protein that binds iron and is involved in the formation of iron-sulfur clusters. Decreased frataxin function leads to decreased iron-sulfur cluster formation, mitochondrial iron accumulation, cytosolic iron depletion, oxidative stress, and mitochondrial dysfunction. Cloning of the disease gene for Friedreich ataxia and elucidation of many aspects of the biochemical defects underlying the disorder have led to several major therapeutic initiatives aimed at increasing frataxin expression, reversing mitochondrial iron accumulation, and alleviating oxidative stress. These initiatives are in preclinical and clinical development and are reviewed herein.

Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia

1997 ◽  
Vol 17 (2) ◽  
pp. 215-217 ◽  
Author(s):  
Agnès Rötig ◽  
Pascale de Lonlay ◽  
Dominique Chretien ◽  
Françoise Foury ◽  
Michel Koenig ◽  
...  
Keyword(s):  
Friedreich Ataxia ◽  
Protein Deficiency ◽  
Mitochondrial Iron

scholarly journals Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia

2012 ◽  
Vol 109 (50) ◽  
pp. 20590-20595 ◽  
Author(s):  
M. Whitnall ◽  
Y. S. Rahmanto ◽  
M. L.- H. Huang ◽  
F. Saletta ◽  
H. C. Lok ◽  
...  
Keyword(s):  
Mouse Model ◽  
Friedreich Ataxia ◽  
Mitochondrial Iron ◽  
Iron Deposits

scholarly journals Ironing out a therapy for Friedreich ataxia

Blood ◽  
2007 ◽  
Vol 110 (1) ◽  
pp. 1-2 ◽  
Author(s):  
Grazia Isaya
Keyword(s):  
Iron Metabolism ◽  
Inborn Error ◽  
Friedreich Ataxia ◽  
Young Patients ◽  
Iron Chelation ◽  
Positive Effects ◽  
Mitochondrial Iron

Boddaert and colleagues show the positive effects of selective iron chelation in young patients with Friedreich ataxia, a devastating inborn error of mitochondrial iron metabolism.

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