Literature DB >> 19101701

Efficacy of idebenone for respiratory failure in a patient with Leigh syndrome: a long-term follow-up study.

Kazuhiro Haginoya1, Shigeaki Miyabayashi, Masahiro Kikuchi, Akira Kojima, Katsuya Yamamoto, Kiyoshi Omura, Mitsugu Uematsu, Naomi Hino-Fukuyo, Soichiro Tanaka, Shigeru Tsuchiya.   

Abstract

Respiratory failure can be the direct cause of death in patients with Leigh syndrome. Unfortunately, no effective treatment strategy is available. Here, we report successful treatment of a patient with Leigh syndrome using idebenone, a derivative of coenzyme Q-10. The patient's brainstem function, especially respiratory function, improved after idebenone treatment. Idebenone may be worth trying in patients with Leigh syndrome.

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Year:  2008        PMID： 19101701     DOI： 10.1016/j.jns.2008.11.008

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


  10 in total

Review 1.  Mitochondrial genetic diseases.

Authors:  Marni J Falk; Neal Sondheimer
Journal:  Curr Opin Pediatr       Date:  2010-12       Impact factor: 2.856

2.  Mitochondrial respiration without ubiquinone biosynthesis.

Authors:  Ying Wang; Siegfried Hekimi
Journal:  Hum Mol Genet       Date:  2013-07-11       Impact factor: 6.150

Review 3.  Diagnosis and treatment of mitochondrial myopathies.

Authors:  Gerald Pfeffer; Patrick F Chinnery
Journal:  Ann Med       Date:  2011-08-25       Impact factor: 4.709

4.  Features of idebenone and related short-chain quinones that rescue ATP levels under conditions of impaired mitochondrial complex I.

Authors:  Michael Erb; Barbara Hoffmann-Enger; Holger Deppe; Michael Soeberdt; Roman H Haefeli; Christian Rummey; Achim Feurer; Nuri Gueven
Journal:  PLoS One       Date:  2012-04-27       Impact factor: 3.240

5.  NQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levels.

Authors:  Roman H Haefeli; Michael Erb; Anja C Gemperli; Dimitri Robay; Isabelle Courdier Fruh; Corinne Anklin; Robert Dallmann; Nuri Gueven
Journal:  PLoS One       Date:  2011-03-31       Impact factor: 3.240

6.  The effects of idebenone on mitochondrial bioenergetics.

Authors:  Valentina Giorgio; Valeria Petronilli; Anna Ghelli; Valerio Carelli; Michela Rugolo; Giorgio Lenaz; Paolo Bernardi
Journal:  Biochim Biophys Acta       Date:  2011-11-04

Review 7.  Border between natural product and drug: comparison of the related benzoquinones idebenone and coenzyme Q10.

Authors:  Nuri Gueven; Krystel Woolley; Jason Smith
Journal:  Redox Biol       Date:  2015-01-14       Impact factor: 11.799

8.  Antioxidant Amelioration of Riboflavin Transporter Deficiency in Motoneurons Derived from Patient-Specific Induced Pluripotent Stem Cells.

Authors:  Chiara Marioli; Valentina Magliocca; Stefania Petrini; Alessia Niceforo; Rossella Borghi; Sara Petrillo; Piergiorgio La Rosa; Fiorella Colasuonno; Tiziana Persichini; Fiorella Piemonte; Keith Massey; Marco Tartaglia; Sandra Moreno; Enrico Bertini; Claudia Compagnucci
Journal:  Int J Mol Sci       Date:  2020-10-07       Impact factor: 5.923

Review 9.  Idebenone: When an antioxidant is not an antioxidant.

Authors:  Nuri Gueven; Pranathi Ravishankar; Rajaraman Eri; Emma Rybalka
Journal:  Redox Biol       Date:  2020-11-25       Impact factor: 11.799

10.  Clinical exome sequencing reveals a mutation in PDHA1 in Leigh syndrome: A case of a Chinese boy with lethal neuropathy.

Authors:  Ke Gong; Li Xie; Zhong-Shi Wu; Xia Xie; Xing-Xing Zhang; Jin-Lan Chen
Journal:  Mol Genet Genomic Med       Date:  2021-03-04       Impact factor: 2.183
  10 in total

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