Literature DB >> 2089142

Ubidecarenone in the treatment of mitochondrial myopathies: a multi-center double-blind trial.

N Bresolin1, C Doriguzzi, C Ponzetto, C Angelini, I Moroni, E Castelli, E Cossutta, A Binda, A Gallanti, S Gabellini.   

Abstract

Forty-four patients with mitochondrial myopathies were treated with Ubidecarenone (CoQ10) for 6 months in an open multi-center trial. No side effects of the drug were observed. Sixteen patients showing at least 25% decrease of post-exercise lactate levels were selected as responders. Responsiveness was apparently not related to CoQ10 level in serum and platelets or to the presence or absence of mtDNA deletions. The responders were treated for a further 3 months with CoQ10 or placebo in the second blind part of the trial; no significant differences were observed between the 2 groups. It is not clear why CoQ10 had therapeutic effects in some patients and not in others with the same clinical presentation and biochemical defect, and we failed to identify candidate responders before treatment. At the dose of CoQ10 used in this study (2 mg/kg/day) the therapy requires a long administration time before a response is seen.

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Year:  1990        PMID: 2089142     DOI: 10.1016/0022-510x(90)90015-f

Source DB:  PubMed          Journal:  J Neurol Sci        ISSN: 0022-510X            Impact factor:   3.181


  20 in total

Review 1.  Clinical mitochondrial genetics.

Authors:  P F Chinnery; N Howell; R M Andrews; D M Turnbull
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2.  Could coenzyme Q10 and L-carnitine be a treatment for diabetes secondary to 3243 mutation of mtDNA?

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Journal:  Diabetologia       Date:  1995-12       Impact factor: 10.122

Review 3.  The mitochondrial myopathy encephalopathy, lactic acidosis with stroke-like episodes (MELAS) syndrome: a review of treatment options.

Authors:  Fernando Scaglia; Jennifer L Northrop
Journal:  CNS Drugs       Date:  2006       Impact factor: 5.749

4.  Coenzyme q10 therapy.

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Journal:  Mol Syndromol       Date:  2014-07

Review 5.  The treatment of congenital lactic acidoses.

Authors:  A A Morris; J V Leonard
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982

Review 6.  Treatment of mitochondrial disease.

Authors:  R W Taylor; P F Chinnery; K M Clark; R N Lightowlers; D M Turnbull
Journal:  J Bioenerg Biomembr       Date:  1997-04       Impact factor: 2.945

7.  Mitochondrial disease patients' perception of dietary supplements' use.

Authors:  Amel Karaa; Joshua Kriger; Johnston Grier; Amy Holbert; John L P Thompson; Sumit Parikh; Michio Hirano
Journal:  Mol Genet Metab       Date:  2016-07-16       Impact factor: 4.797

Review 8.  Coenzyme Q10 depletion in medical and neuropsychiatric disorders: potential repercussions and therapeutic implications.

Authors:  Gerwyn Morris; George Anderson; Michael Berk; Michael Maes
Journal:  Mol Neurobiol       Date:  2013-06-13       Impact factor: 5.590

9.  Effect of high-dose vitamins, coenzyme Q and high-fat diet in paediatric patients with mitochondrial diseases.

Authors:  J Panetta; L J Smith; A Boneh
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

Review 10.  Treatment of hereditary optic neuropathies.

Authors:  Nancy J Newman
Journal:  Nat Rev Neurol       Date:  2012-09-04       Impact factor: 42.937

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