Sensitivity of FRDA lymphoblasts to salts of transition metal ions.
Sensitivity of FRDA lymphoblasts to salts of transition metal ions.
复制标题
FRDA 淋巴母细胞对过渡金属离子盐的敏感性。
DOI：
10.1089/15230860050192233
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发表时间：
2000
影响因子：
6.6
通讯作者：
Cortopassi,G
中科院分区：
文献类型：
--
作者：
Wong,A;Yang,J;Danielson,S;Gellera,C;Taroni,F;Cortopassi,G
Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease resulting from decreased expression of the nuclear-encoded mitochondrial protein, frataxin. FRDA patients have characteristic iron deposits and dysfunction of mitochondrial enzymes in the heart. Inactivation of the frataxin homologue in yeast causes dysregulation of both mitochondrial iron levels and iron export. Previously, we have observed sensitivity of FRDA fibroblasts to FeCl3and hydrogen peroxide, results consistent with the hypothesis that FRDA cells may experience increased Fenton chemistry. To determine whether the sensitivity of FRDA cells to transition metal ions is a general or specific property, we have compared the sensitivity of lymphoblasts from FRDA patients and healthy controls to the transition metal salts CoCl2, CuSO4FeCl3FeSO4, MnCl2, and ZnCl2. FRDA lymphoblasts were significantly more sensitive to FeCl3and MnCl2than control cells. However, there were no significant differences observed in sensitivity to CoCl2, CuSO4, FeSO4and ZnCl2in the concentration ranges studied. Thus, the sensitivity of FRDA lymphoblasts exposed to transition metals appears to be specific, and could be relevant to the pathophysiological mechanism, which is discussed.
