Monitoring cardiac function during idebenone therapy in Friedreich's ataxia

D Salvo Giovanni, P Valeria, F Bahaa… - Current …, 2015 - benthamdirect.com
Friedreich's ataxia (FA) is associated with progressive cardiac hypertrophy resulting from a
genetic abnormality in the frataxin gene. Cardiac involvement is the most common cause of …

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

AO Hausse, Y Aggoun, D Bonnet, D Sidi, A Munnich… - Heart, 2002 - heart.bmj.com
Background: Friedreich's ataxia encodes a protein of unknown function, frataxin. The loss of
frataxin is caused by a large GAA trinucleotide expansion in the first intron of the gene …

Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia

P Rustin, A Rötig, A Munnich, D Sidi - Free radical research, 2002 - Taylor & Francis
Friedreich's ataxia (FRDA) is a neuro-degenerative disease causing limb and gait ataxia
and hypertrophic cardiomyopathy. It results from a triplet expansion in the first intron of the …

Idebenone in patients with Friedreich ataxia

L Schöls, M Vorgerd, M Schillings, G Skipka… - Neuroscience letters, 2001 - Elsevier
Friedreich ataxia (FA), the most common form of degenerative ataxia, is thought to be
caused by respiratory deficiency due to mitochondrial iron accumulation and oxidative …

FRIEDREICH'S ATAXIA

M Čačić Hribljan, M Jurin - Paediatria Croatica, 2011 - hrcak.srce.hr
Sažetak Friedreich's ataxia (FRDA) is the most common autosomal recessive ataxia. This
neurodegenerative disease is caused by expansion of a GAA triplet repeat located within …

Cardiomyopathy in Friedreich Ataxia: Exemplifying the Challenges Faced by Cardiologists in the Management of Rare Diseases

M Kvistholm Jensen, H Bundgaard - Circulation, 2012 - Am Heart Assoc
Friedreich ataxia (FA) is an autosomal recessively inher-ited neurodegenerative disease
that most often presents in childhood or in young adulthood. A substantial proportion of …

[PDF][PDF] Cardiomyopathy in Friedreich Ataxia

MK Jensen, H Bundgaard - Circulation, 2012 - scholar.archive.org
Friedreich ataxia (FA) is an autosomal recessively inher-ited neurodegenerative disease
that most often presents in childhood or in young adulthood. A substantial proportion of …

Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial

NA Di Prospero, A Baker, N Jeffries… - The Lancet …, 2007 - thelancet.com
Background Friedreich's ataxia (FA) is a progressive, multisystem, degenerative disorder
caused by a reduction in frataxin. Loss of frataxin results in mitochondrial dysfunction and …

Idebenone: an emerging therapy for Friedreich ataxia

T Meier, G Buyse - Journal of neurology, 2009 - Springer
This paper reviews the history and pre-clinical development of idebenone and summarises
the results of clinical studies, published from 1999 to 2008, on the use of idebenone in the …

Idebenone in Friedreich's ataxia

C Tonon, R Lodi - Expert opinion on pharmacotherapy, 2008 - Taylor & Francis
Background: Friedreich's ataxia is an autosomal recessive neurodegenerative disease
where impaired mitochondrial function and excessive production of free radicals play a …