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Redistribution of accumulated cell iron: a modality of chelation with therapeutic implications.
Sohn YS, Breuer W, Munnich A, Cabantchik ZI. Sohn YS, et al. Blood. 2008 Feb 1;111(3):1690-9. doi: 10.1182/blood-2007-07-102335. Epub 2007 Nov 1. Blood. 2008. PMID: 17975016
We explored the possibility of using deferiprone, a membrane-permeant iron chelator in clinical use, to capture labile iron accumulated in specific organelles of cardiomyocytes and macrophages and convey it to other locations for physiologic reuse. ...
We explored the possibility of using deferiprone, a membrane-permeant iron chelator in clinical use, to capture labile iron accumulat …
Regional siderosis: a new challenge for iron chelation therapy.
Cabantchik ZI, Munnich A, Youdim MB, Devos D. Cabantchik ZI, et al. Front Pharmacol. 2013 Dec 31;4:167. doi: 10.3389/fphar.2013.00167. Front Pharmacol. 2013. PMID: 24427136 Free PMC article. Review.
For this purpose we introduced a conservative mode of iron chelation of dual activity, one based on scavenging labile metal but also redeploying it to cell acceptors or to physiological transferrin. ...The proposed deferiprone-based therapy has provided a paradigm f …
For this purpose we introduced a conservative mode of iron chelation of dual activity, one based on scavenging labile metal but also …
Iron redistribution as a therapeutic strategy for treating diseases of localized iron accumulation.
Kakhlon O, Breuer W, Munnich A, Cabantchik ZI. Kakhlon O, et al. Can J Physiol Pharmacol. 2010 Mar;88(3):187-96. doi: 10.1139/Y09-128. Can J Physiol Pharmacol. 2010. PMID: 20393584 Review.
Defective iron utilization leading to either systemic or regional misdistribution of the metal has been identified as a critical feature of several different disorders. ...DFP was applied to a cell model of FRDA, a paradigm of a disorder etiologically …
Defective iron utilization leading to either systemic or regional misdistribution of the metal has been identified as a critical feat …
Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation.
Kakhlon O, Manning H, Breuer W, Melamed-Book N, Lu C, Cortopassi G, Munnich A, Cabantchik ZI. Kakhlon O, et al. Blood. 2008 Dec 15;112(13):5219-27. doi: 10.1182/blood-2008-06-161919. Epub 2008 Sep 16. Blood. 2008. PMID: 18796625
Friedreich ataxia (FRDA), a deficiency in the mitochondrial iron-chaperone frataxin, results in defective use of iron and its misdistribution between mitochondria and cytosol. We assessed the possibility of functionally correcting the cellular properties affected by fratax …
Friedreich ataxia (FRDA), a deficiency in the mitochondrial iron-chaperone frataxin, results in defective use of iron and its misdist …
Selective iron chelation in Friedreich ataxia: biologic and clinical implications.
Boddaert N, Le Quan Sang KH, Rötig A, Leroy-Willig A, Gallet S, Brunelle F, Sidi D, Thalabard JC, Munnich A, Cabantchik ZI. Boddaert N, et al. Blood. 2007 Jul 1;110(1):401-8. doi: 10.1182/blood-2006-12-065433. Epub 2007 Mar 22. Blood. 2007. PMID: 17379741 Clinical Trial.
We assessed the possibility of reducing brain iron accumulation in Friedreich ataxia patients with a membrane-permeant chelator capable of shuttling chelated iron from cells to transferrin, using regimens suitable for patients with no systemic iron overload. ...To our know …
We assessed the possibility of reducing brain iron accumulation in Friedreich ataxia patients with a membrane-permeant chelator capab …
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia.
Hausse AO, Aggoun Y, Bonnet D, Sidi D, Munnich A, Rötig A, Rustin P. Hausse AO, et al. Heart. 2002 Apr;87(4):346-9. doi: 10.1136/heart.87.4.346. Heart. 2002. PMID: 11907009 Free PMC article. Clinical Trial.
BACKGROUND: Friedreich's ataxia encodes a protein of unknown function, frataxin. The loss of frataxin is caused by a large GAA trinucleotide expansion in the first intron of the gene, resulting in deficiency of a Krebs cycle enzyme, aconitase, and of three mi …
BACKGROUND: Friedreich's ataxia encodes a protein of unknown function, frataxin. The loss of frataxin is caused by a large GAA …
Molar tooth sign and superior vermian dysplasia: a radiological, clinical, and genetic study.
Romano S, Boddaert N, Desguerre I, Hubert L, Salomon R, Seidenwurm D, Bahi-Buisson N, Nabbout R, Sonigo P, Lyonnet S, Brunelle F, Munnich A, de Lonlay P. Romano S, et al. Neuropediatrics. 2006 Feb;37(1):42-5. doi: 10.1055/s-2006-923838. Neuropediatrics. 2006. PMID: 16541367
We have identified a group of 13 patients with a homogeneous radiological pattern at MRI consisting of the molar tooth sign (MTS) and superior vermian dysplasia. ...The present study suggests that the syndrome associating MTS and dysplasia of the superior vermis of …
We have identified a group of 13 patients with a homogeneous radiological pattern at MRI consisting of the molar tooth sign (M …
Cerebellar ataxia and mental retardation in a child with an inherited satellited chromosome 4q.
Faivre L, Radford I, Viot G, Edery P, Munnich A, Tardieu M, Vekemans M. Faivre L, et al. Ann Genet. 2000 Jan-Mar;43(1):35-8. doi: 10.1016/s0003-3995(00)00016-2. Ann Genet. 2000. PMID: 10818219
We identified a case of familial satellited chromosome 4q through a child with cerebellar ataxia and mental retardation. No loss of genetic material could be demonstrated at the molecular level but other possible mechanisms of this association are discussed. ...
We identified a case of familial satellited chromosome 4q through a child with cerebellar ataxia and mental retardation. No lo …
[Friedreich's ataxia and mitochondria: the puzzle reconstructed].
Rötig A, Munnich A, Rustin P. Rötig A, et al. Arch Pediatr. 1999;6 Suppl 2:498s-499s. doi: 10.1016/s0929-693x(99)80517-x. Arch Pediatr. 1999. PMID: 10370585 French. No abstract available.
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