Sensitivity of FRDA lymphoblasts to salts of transition metal ions

A. Wong, J. Yang, S. Danielson, C. Gellera, F. Taroni, G. Cortopassi

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

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 FeCl3 and 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, CuSO4 FeCl3 FeSO4, MnCl2, and ZnCl2. FRDA lymphoblasts were significantly more sensitive to FeCl3 and MnCl2 than control cells. However, there were no significant differences observed in sensitivity to CoCl2, CuSO4, FeSO4 and ZnCl2 in 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.

Original languageEnglish
Pages (from-to)461-465
Number of pages5
JournalAntioxidants and Redox Signaling
Volume2
Issue number3
Publication statusPublished - 2000

Fingerprint

Friedreich Ataxia
Transition metals
Metal ions
Salts
Metals
Ions
Iron deposits
Iron
Neurodegenerative diseases
Mitochondrial Proteins
Fibroblasts
Yeast
Hydrogen Peroxide
Enzymes
Neurodegenerative Diseases
manganese chloride
frataxin
Yeasts

ASJC Scopus subject areas

  • Biochemistry

Cite this

Sensitivity of FRDA lymphoblasts to salts of transition metal ions. / Wong, A.; Yang, J.; Danielson, S.; Gellera, C.; Taroni, F.; Cortopassi, G.

In: Antioxidants and Redox Signaling, Vol. 2, No. 3, 2000, p. 461-465.

Research output: Contribution to journalArticle

Wong, A, Yang, J, Danielson, S, Gellera, C, Taroni, F & Cortopassi, G 2000, 'Sensitivity of FRDA lymphoblasts to salts of transition metal ions', Antioxidants and Redox Signaling, vol. 2, no. 3, pp. 461-465.
Wong, A. ; Yang, J. ; Danielson, S. ; Gellera, C. ; Taroni, F. ; Cortopassi, G. / Sensitivity of FRDA lymphoblasts to salts of transition metal ions. In: Antioxidants and Redox Signaling. 2000 ; Vol. 2, No. 3. pp. 461-465.
@article{4c3f9908b0a24fb69d7febbeada37864,
title = "Sensitivity of FRDA lymphoblasts to salts of transition metal ions",
abstract = "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 FeCl3 and 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, CuSO4 FeCl3 FeSO4, MnCl2, and ZnCl2. FRDA lymphoblasts were significantly more sensitive to FeCl3 and MnCl2 than control cells. However, there were no significant differences observed in sensitivity to CoCl2, CuSO4, FeSO4 and ZnCl2 in 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.",
author = "A. Wong and J. Yang and S. Danielson and C. Gellera and F. Taroni and G. Cortopassi",
year = "2000",
language = "English",
volume = "2",
pages = "461--465",
journal = "Antioxidants and Redox Signaling",
issn = "1523-0864",
publisher = "Mary Ann Liebert Inc.",
number = "3",

}

TY - JOUR

T1 - Sensitivity of FRDA lymphoblasts to salts of transition metal ions

AU - Wong, A.

AU - Yang, J.

AU - Danielson, S.

AU - Gellera, C.

AU - Taroni, F.

AU - Cortopassi, G.

PY - 2000

Y1 - 2000

N2 - 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 FeCl3 and 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, CuSO4 FeCl3 FeSO4, MnCl2, and ZnCl2. FRDA lymphoblasts were significantly more sensitive to FeCl3 and MnCl2 than control cells. However, there were no significant differences observed in sensitivity to CoCl2, CuSO4, FeSO4 and ZnCl2 in 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.

AB - 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 FeCl3 and 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, CuSO4 FeCl3 FeSO4, MnCl2, and ZnCl2. FRDA lymphoblasts were significantly more sensitive to FeCl3 and MnCl2 than control cells. However, there were no significant differences observed in sensitivity to CoCl2, CuSO4, FeSO4 and ZnCl2 in 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.

UR - http://www.scopus.com/inward/record.url?scp=0033751576&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0033751576&partnerID=8YFLogxK

M3 - Article

C2 - 11229359

AN - SCOPUS:0033751576

VL - 2

SP - 461

EP - 465

JO - Antioxidants and Redox Signaling

JF - Antioxidants and Redox Signaling

SN - 1523-0864

IS - 3

ER -