| 19997898 |
Richardson DR, Huang ML, Whitnall M, Becker EM, Ponka P, Rahmanto YS: The ins and outs of mitochondrial iron-loading: the metabolic defect in Friedreich's ataxia. J Mol Med. 2010 Apr;88(4):323-9. Epub 2009 Dec 9.
This stimulation of iron uptake probably attempts to rescue the deficit in mitochondrial iron metabolism that is due to downregulation of mitochondrial iron utilization, namely, heme and iron-sulfur cluster (ISC) synthesis and also iron storage (mitochondrial ferritin). |
81(1,1,1,1) |
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| 19805308 |
Huang ML, Becker EM, Whitnall M, Rahmanto YS, Ponka P, Richardson DR: Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant. Proc Natl Acad Sci U S A. 2009 Sep 22;106(38):16381-6. Epub 2009 Sep 4.
Using cardiac tissues, we demonstrate that frataxin deficiency leads to down-regulation of key molecules involved in 3 mitochondrial utilization pathways: iron-sulfur cluster (ISC) synthesis (iron-sulfur cluster scaffold protein1/2 and the cysteine desulferase Nfs1), mitochondrial iron storage (mitochondrial ferritin), and heme synthesis (5-aminolevulinate dehydratase, coproporphyrinogen oxidase, hydroxymethylbilane synthase, uroporphyrinogen III synthase, and ferrochelatase). |
31(0,1,1,1) |
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