Idebenone in Friedreich's ataxia.

Tonon C, Lodi R.

UniversitÓ di Bologna,
Dipartimento di Medicina Interna,
dell'Invecchiamento e Malattie Nefrologiche,
Azienda Ospedaliero-Universitaria di Bologna,
Via Massarenti 9, 40138 Bologna, Italy.

ABSTRACT

BACKGROUND: Friedreich's ataxia is an autosomal recessive neurodegenerative disease where impaired mitochondrial function and excessive production of free radicals play a central pathogenetic role. Idebenone, a synthetic analogue of coenzyme Q, is a powerful antioxidant that was first administrated to Friedreich's ataxia patients less than 10 years ago.

OBJECTIVE: The aim of this study was to evaluate the efficacy of idebenone administration and define the optimal dosage.

METHODS: A critical evaluation of all open and double-blinded idebenone trials in Friedreich's ataxia patients was undertaken.

RESULTS/CONCLUSIONS: Idebenone is well tolerated in paediatric and adult patients. Most trials demonstrated a positive effect on cardiac hypertrophy. The neurological function is in general not modified in adult patients, but a dose-dependent effect was demonstrated in young Friedreich's ataxia patients. Further double-blinded high-dose trials should evaluate idebenone in Friedreich's ataxia early in the disease course.

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Friedreich's ataxia.
Idebenone  and reduced cardiac hypertrophy in Friedreich's ataxia.
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MELAS
Idebenone  improves cerebral mitochondrial oxidative metabolism in MELAS
Idebenone  in young patients with mitochondrial encephalomyopathy (MELAS)
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Idebenone  and Mitochondrial encephalomyopathy (MELAS)
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Cardiomyopathy
Idebenone  improvement in mitochondrial cardiomyopathy following treatment with idebenone

Idebenone   effect of idebenone on cardiomyopathy in Friedreich's ataxia
 .

Leber hereditary optic neuropathy (LHON)
Idebenone  does it shorten the time to visual recovery in Leber hereditary optic neuropathy?
Idebenone  improvement in a patient with Leber's hereditary optic neuropathy (LHON)

Misc.
Idebenone  use in age-related cognitive disorders

Idebenone  protects hippocampal neurons against beta amyloid neurotoxicity

Idebenone 
functions as a potent protective hepatocyte antioxidant

Idebenone 
neuroprotective effects of idebenone can be attributed to its antioxidant ability

Idebenone 
inhibition of brain mitochondrial swelling

Idebenone 
suppression of cold ischemia/reperfusion injury of liver endothelium

Idebenone 
ability to eliminate a nitroxide radical in the rat

Idebenone  interaction with respiratory complexes of heart mitochondria

Idebenone  effect on serotonin release and seroton receptors in rats

Idebenone  protects hepatic microsomes against damage in organ preservation solutions

Idebenone 
protects against toxicity induced by low density lipoprotein

Idebenone  protects against disorders due to cerebral hypoxia or ischemia

Idebenone  protects against toxicity induced by low density lipoprotein

Idebenone  attenuates neuronal degeneration induced by excitotoxins

Idebenone  augments long-term potentiation in hippocampal slices in the guinea pig

Idebenone
effects on lipid peroxidation in erythrocytes of stroke-prone, hypertensive rats

Idebenone  brain distribution and its effect on local cerebral glucose utilization in rats

Idebenone  effects on acetylcholine levels in the brains of rats with cerebral ischemia

Idebenone  protective effect of idebenone against hypoxia in mice

Idebenone  improves learning and memory impairment induced in rats

Idebenone  effects of idebenone on neurological deficits in stroke-prone rats

Idebenone
  effects on memory induced impairment in rats

Idebenone  effects of idebenone on cerebral blood flow in rats with cerebral ischemia

Idebenone 
inhibition of brain mitochondrial swelling by idebenone

Idebenone 
inhibition of platelet aggregation by idebenone

Idebenone  effects of idebenone on monoamine metabolites of patients with dementia

Idebenone 
effects of idebenone on metabolism of monoamines and cyclic AMP formation

Idebenone
  effects on impairment of radial maze learning in cerebral embolized rats

Idebenone  effect of idebenone on lipid peroxidation in rat brain homogenate

Idebenone  effects on muricidal behavior in rats with raphe lesions

Idebenone  inhibitory effect of idebenone on vascular lesions in hypertensive rats

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