Protein Information

Name FRDA
Synonyms CyaY; FA; FARR; FRDA; FXN; Frataxin; Friedreich ataxia protein; X25…

Compound Information

Name ferrous sulfate
CAS sulfuric acid iron(2+) salt (1:1)

Reference List

PubMed Abstract RScore(About this table)
12915401 Pastore A, Tozzi G, Gaeta LM, Bertini E, Serafini V, Di Cesare S, Bonetto V, Casoni F, Carrozzo R, Federici G, Piemonte F: Actin glutathionylation increases in fibroblasts of patients with Friedreich's ataxia: a potential role in the pathogenesis of the disease. J Biol Chem. 2003 Oct 24;278(43):42588-95. Epub 2003 Aug 11.


Increasing evidence suggests that iron-mediated oxidative stress might underlie the development of neurodegeneration in Friedreich's ataxia (FRDA), an autosomal recessive ataxia caused by decreased expression of frataxin, a protein implicated in iron metabolism.
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12022884 Gakh O, Adamec J, Gacy AM, Twesten RD, Owen WG, Isaya G: Physical evidence that yeast frataxin is an iron storage protein. Biochemistry. 2002 May 28;41(21):6798-804.

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14609915 Alldredge CD, Schlieve CR, Miller NR, Levin LA: Pathophysiology of the optic neuropathy associated with Friedreich ataxia. . Arch Ophthalmol. 2003 Nov;121(11):1582-5.


CONCLUSION: We hypothesize that decreased expression of frataxin, the mutated gene in Friedreich ataxia, could cause an optic neuropathy by increasing the sensitivity of retinal ganglion cells to oxidative stress.
1(0,0,0,1) Details