scholarly journals A pathogenic role for histone H3 copper reductase activity in a yeast model of Friedreich’s ataxia

2021 ◽  
Vol 7 (51) ◽  
Author(s):  
Oscar A. Campos ◽  
Narsis Attar ◽  
Chen Cheng ◽  
Maria Vogelauer ◽  
Nathan V. Mallipeddi ◽  
...  
Keyword(s):  
Reductase Activity ◽  
Histone H3 ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Pathogenic Role ◽  
Yeast Model

scholarly journals A pathogenic role for histone H3 copper reductase activity in a yeast model of Friedreich's Ataxia

2021 ◽  
Author(s):  
Oscar A Campos ◽  
Narsis Attar ◽  
Nathan V Mallipeddi ◽  
Chen Cheng ◽  
Maria Vogelauer ◽  
...  
Keyword(s):  
Enzyme Activity ◽  
Human Disease ◽  
Reductase Activity ◽  
Histone H3 ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Pathogenic Role ◽  
Growth Defects ◽  
Iron Sulfur ◽  
Cellular Copper

Disruptions to iron-sulfur (Fe-S) clusters, essential cofactors for a broad range of proteins, cause widespread cellular defects resulting in human disease. An underappreciated source of damage to Fe-S clusters are cuprous (Cu1+) ions. Since histone H3 enzymatically produces Cu1+ to support copper-dependent functions, we asked whether this activity could become detrimental to Fe-S clusters. Here, we report that histone H3-mediated Cu1+ toxicity is a major determinant of cellular Fe-S cluster quotient. Inadequate Fe-S cluster supply, either due to diminished assembly as occurs in Friedreich's Ataxia or defective distribution, causes severe metabolic and growth defects in S. cerevisiae. Decreasing Cu1+ abundance, through attenuation of histone cupric reductase activity or depletion of total cellular copper, restored Fe-S cluster-dependent metabolism and growth. Our findings reveal a novel interplay between chromatin and mitochondria in Fe-S cluster homeostasis, and a potential pathogenic role for histone enzyme activity and Cu1+ in diseases with Fe-S cluster dysfunction.

Spectroscopic characterization of iron deposits in a yeast model(YFH1-) of Friedreich's Ataxia

2003 ◽  
Vol 96 (1) ◽  
pp. 189
Author(s):  
Berthold F. Matzanke ◽  
Emanuel Lesuisse ◽  
Volker Schünemann ◽  
Alfred X. Trautwein ◽  
Wolfram Meyer-Klaucke
Keyword(s):  
Spectroscopic Characterization ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Iron Deposits ◽  
Yeast Model

scholarly journals Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia

2008 ◽  
Vol 17 (18) ◽  
pp. 2790-2802 ◽  
Author(s):  
F. Auchere ◽  
R. Santos ◽  
S. Planamente ◽  
E. Lesuisse ◽  
J.-M. Camadro
Keyword(s):  
Friedreich’S Ataxia ◽  
Redox Status ◽  
Friedreich's Ataxia ◽  
Yeast Model

Cardiomyopathy in Friedreich's ataxia: assessment by echocardiography and cardiac magnetic resonance imaging

2005 ◽  
Vol 32 (S 4) ◽  
Author(s):  
C Meyer ◽  
G Schmid ◽  
S Görlitz ◽  
M Schillings ◽  
I Wilhelm ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Cardiac Magnetic Resonance Imaging ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Resonance Imaging

Otoneurological Findings in Friedreich's Ataxia and Other Inherited Neuropathies

1986 ◽  
Vol 25 (2) ◽  
pp. 84-91 ◽  
Author(s):  
E. Cassandro ◽  
F. Mosca ◽  
L. Sequino ◽  
F. A. De Falco ◽  
G. Campanella
Keyword(s):  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Inherited Neuropathies

scholarly journals Echocardiographic Findings in Friedreich's Ataxia

1976 ◽  
Vol 3 (4) ◽  
pp. 329-332 ◽  
Author(s):  
H.F. Gattiker ◽  
A. Davignon ◽  
A. Bozio ◽  
J. Batlle-Diaz ◽  
G. Geoffroy ◽  
...  
Keyword(s):  
Friedreich’S Ataxia ◽  
Left Ventricular ◽  
Friedreich's Ataxia ◽  
Ventricular Free Wall ◽  
Slight Reduction ◽  
Free Wall ◽  
Left Ventricular Free Wall ◽  
Echocardiographic Examination ◽  
Septal Hypertrophy ◽  
Echocardiographic Findings

SUMMARY:Echocardiographic examination of 21 patients with Friedreich's ataxia (age 7 to 28 years) showed cardiac abnormalities in 90% of the cases. They were characterized by varying degrees of septal hypertrophy in 81%, left ventricular free wall hypertrophy in 61%, and a slight reduction of left ventricular internal dimension in 57% of the cases. Asymmetric septal hypertrophy (ASH) with a septal/left ventricular free wall ratio of over 1.3 was found in 29% of the cases, and systolic anterior motion (SAM) of the mitral valve in three patients. Two other patients showed evidence of a different type of cardiomyopathy with marked symmetric left ventricular hypertrophy and marked left ventricular enlargement.

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