scholarly journals A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxia

2015 ◽  
Vol 8 (3) ◽  
pp. 225-235 ◽  
Author(s):  
S. Anjomani Virmouni ◽  
V. Ezzatizadeh ◽  
C. Sandi ◽  
M. Sandi ◽  
S. Al-Mahdawi ◽  
...  
Keyword(s):  
Mouse Model ◽  
Friedreich’S Ataxia ◽  
Repeat Expansion ◽  
Friedreich's Ataxia ◽  
Gaa Repeat Expansion

The GAA repeat expansion in intron 1 of the frataxin gene is related to the severity of cardiac manifestation in patients with Friedreich's ataxia

2000 ◽  
Vol 78 (11) ◽  
pp. 626-632 ◽  
Author(s):  
Nana Bit-Avragim ◽  
Andreas Perrot ◽  
Ludger Schöls ◽  
Cornelia Hardt ◽  
Friedmar R. Kreuz ◽  
...  
Keyword(s):  
Friedreich’S Ataxia ◽  
Repeat Expansion ◽  
Friedreich's Ataxia ◽  
Intron 1 ◽  
Gaa Repeat Expansion ◽  
Cardiac Manifestation ◽  
Frataxin Gene

Friedreich's ataxia GAA repeat expansion in patients with recessive or sporadic ataxia

Neurology ◽  
1997 ◽  
Vol 49 (4) ◽  
pp. 1004-1009 ◽  
Author(s):  
D. H. Geschwind ◽  
S. Perlman ◽  
W. W. Grody ◽  
M. Telatar ◽  
L. Montermini ◽  
...  
Keyword(s):  
Friedreich’S Ataxia ◽  
Repeat Expansion ◽  
Friedreich's Ataxia ◽  
Gaa Repeat Expansion ◽  
Sporadic Ataxia

scholarly journals A GAA repeat expansion reporter model of Friedreich's ataxia recapitulates the genomic context and allows rapid screening of therapeutic compounds

2013 ◽  
Vol 22 (25) ◽  
pp. 5173-5187 ◽  
Author(s):  
M. M. P. Lufino ◽  
A. M. Silva ◽  
A. H. Nemeth ◽  
J. Alegre-Abarrategui ◽  
A. J. Russell ◽  
...  
Keyword(s):  
Friedreich’S Ataxia ◽  
Repeat Expansion ◽  
Friedreich's Ataxia ◽  
Rapid Screening ◽  
Genomic Context ◽  
Gaa Repeat Expansion ◽  
Therapeutic Compounds

scholarly journals Two New Pimelic Diphenylamide HDAC Inhibitors Induce Sustained Frataxin Upregulation in Cells from Friedreich's Ataxia Patients and in a Mouse Model

PLoS ONE ◽  
2010 ◽  
Vol 5 (1) ◽  
pp. e8825 ◽  
Author(s):  
Myriam Rai ◽  
Elisabetta Soragni ◽  
C. James Chou ◽  
Glenn Barnes ◽  
Steve Jones ◽  
...  
Keyword(s):  
Mouse Model ◽  
Hdac Inhibitors ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
In Cells

Stress-Induced Mouse Model of the Cardiac Manifestations of Friedreich's Ataxia Corrected by AAV-mediated Gene Therapy

2020 ◽  
Vol 31 (15-16) ◽  
pp. 819-827 ◽  
Author(s):  
Christiana O. Salami ◽  
Katie Jackson ◽  
Clarisse Jose ◽  
Laith Alyass ◽  
Georges-Ibrahim Cisse ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Mouse Model ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Cardiac Manifestations

scholarly journals Hairpin Formation in Friedreich's Ataxia Triplet Repeat Expansion

2002 ◽  
Vol 278 (4) ◽  
pp. 2425-2431 ◽  
Author(s):  
Brooke L. Heidenfelder ◽  
Alexander M. Makhov ◽  
Michael D. Topal
Keyword(s):  
Friedreich’S Ataxia ◽  
Repeat Expansion ◽  
Friedreich's Ataxia ◽  
Triplet Repeat ◽  
Triplet Repeat Expansion ◽  
Hairpin Formation

scholarly journals A TAT–Frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model

2011 ◽  
Vol 21 (6) ◽  
pp. 1230-1247 ◽  
Author(s):  
Piyush M. Vyas ◽  
Wendy J. Tomamichel ◽  
P. Melanie Pride ◽  
Clifford M. Babbey ◽  
Qiujuan Wang ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fusion Protein ◽  
Cardiac Function ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia
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