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Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency.
Biochim Biophys Acta. 2014 Jul; 1842(7):893-901.BB

Abstract

Coenzyme Q10 (CoQ10) deficiency (MIM 607426) causes a mitochondrial syndrome with variability in the clinical presentations. Patients with CoQ10 deficiency show inconsistent responses to oral ubiquinone-10 supplementation, with the highest percentage of unsuccessful results in patients with neurological symptoms (encephalopathy, cerebellar ataxia or multisystemic disease). Failure in the ubiquinone-10 treatment may be the result of its poor absorption and bioavailability, which may be improved by using different pharmacological formulations. In a mouse model (Coq9(X/X)) of mitochondrial encephalopathy due to CoQ deficiency, we have evaluated oral supplementation with water-soluble formulations of reduced (ubiquinol-10) and oxidized (ubiquinone-10) forms of CoQ10. Our results show that CoQ10 was increased in all tissues after supplementation with ubiquinone-10 or ubiquinol-10, with the tissue levels of CoQ10 with ubiquinol-10 being higher than with ubiquinone-10. Moreover, only ubiquinol-10 was able to increase the levels of CoQ10 in mitochondria from cerebrum of Coq9(X/X) mice. Consequently, ubiquinol-10 was more efficient than ubiquinone-10 in increasing the animal body weight and CoQ-dependent respiratory chain complex activities, and reducing the vacuolization, astrogliosis and oxidative damage in diencephalon, septum-striatum and, to a lesser extent, in brainstem. These results suggest that water-soluble formulations of ubiquinol-10 may improve the efficacy of CoQ10 therapy in primary and secondary CoQ10 deficiencies, other mitochondrial diseases and neurodegenerative diseases.

Authors+Show Affiliations

Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada, Spain; Instituto de Biotecnología, Centro de Investigación Biomédica, Parque Tecnológico de Ciencias de la Salud, Armilla, Granada, Spain.Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada, Spain; Instituto de Biotecnología, Centro de Investigación Biomédica, Parque Tecnológico de Ciencias de la Salud, Armilla, Granada, Spain.Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada, Spain; Instituto de Biotecnología, Centro de Investigación Biomédica, Parque Tecnológico de Ciencias de la Salud, Armilla, Granada, Spain.Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada, Spain; Instituto de Biotecnología, Centro de Investigación Biomédica, Parque Tecnológico de Ciencias de la Salud, Armilla, Granada, Spain.Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada, Spain; Instituto de Biotecnología, Centro de Investigación Biomédica, Parque Tecnológico de Ciencias de la Salud, Armilla, Granada, Spain.Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada, Spain; Instituto de Biotecnología, Centro de Investigación Biomédica, Parque Tecnológico de Ciencias de la Salud, Armilla, Granada, Spain.Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada, Spain; Instituto de Biotecnología, Centro de Investigación Biomédica, Parque Tecnológico de Ciencias de la Salud, Armilla, Granada, Spain. Electronic address: luisca@ugr.es.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

24576561

Citation

García-Corzo, Laura, et al. "Ubiquinol-10 Ameliorates Mitochondrial Encephalopathy Associated With CoQ Deficiency." Biochimica Et Biophysica Acta, vol. 1842, no. 7, 2014, pp. 893-901.
García-Corzo L, Luna-Sánchez M, Doerrier C, et al. Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency. Biochim Biophys Acta. 2014;1842(7):893-901.
García-Corzo, L., Luna-Sánchez, M., Doerrier, C., Ortiz, F., Escames, G., Acuña-Castroviejo, D., & López, L. C. (2014). Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency. Biochimica Et Biophysica Acta, 1842(7), 893-901. https://doi.org/10.1016/j.bbadis.2014.02.008
García-Corzo L, et al. Ubiquinol-10 Ameliorates Mitochondrial Encephalopathy Associated With CoQ Deficiency. Biochim Biophys Acta. 2014;1842(7):893-901. PubMed PMID: 24576561.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency. AU - García-Corzo,Laura, AU - Luna-Sánchez,Marta, AU - Doerrier,Carolina, AU - Ortiz,Francisco, AU - Escames,Germaine, AU - Acuña-Castroviejo,Darío, AU - López,Luis C, Y1 - 2014/02/24/ PY - 2013/12/19/received PY - 2014/01/30/revised PY - 2014/02/17/accepted PY - 2014/3/1/entrez PY - 2014/3/1/pubmed PY - 2014/7/25/medline KW - CoQ(10) deficiency KW - Mitochondrial encephalopathy KW - Mouse model KW - Ubiquinol-10 SP - 893 EP - 901 JF - Biochimica et biophysica acta JO - Biochim Biophys Acta VL - 1842 IS - 7 N2 - Coenzyme Q10 (CoQ10) deficiency (MIM 607426) causes a mitochondrial syndrome with variability in the clinical presentations. Patients with CoQ10 deficiency show inconsistent responses to oral ubiquinone-10 supplementation, with the highest percentage of unsuccessful results in patients with neurological symptoms (encephalopathy, cerebellar ataxia or multisystemic disease). Failure in the ubiquinone-10 treatment may be the result of its poor absorption and bioavailability, which may be improved by using different pharmacological formulations. In a mouse model (Coq9(X/X)) of mitochondrial encephalopathy due to CoQ deficiency, we have evaluated oral supplementation with water-soluble formulations of reduced (ubiquinol-10) and oxidized (ubiquinone-10) forms of CoQ10. Our results show that CoQ10 was increased in all tissues after supplementation with ubiquinone-10 or ubiquinol-10, with the tissue levels of CoQ10 with ubiquinol-10 being higher than with ubiquinone-10. Moreover, only ubiquinol-10 was able to increase the levels of CoQ10 in mitochondria from cerebrum of Coq9(X/X) mice. Consequently, ubiquinol-10 was more efficient than ubiquinone-10 in increasing the animal body weight and CoQ-dependent respiratory chain complex activities, and reducing the vacuolization, astrogliosis and oxidative damage in diencephalon, septum-striatum and, to a lesser extent, in brainstem. These results suggest that water-soluble formulations of ubiquinol-10 may improve the efficacy of CoQ10 therapy in primary and secondary CoQ10 deficiencies, other mitochondrial diseases and neurodegenerative diseases. SN - 0006-3002 UR - https://www.unboundmedicine.com/medline/citation/24576561/Ubiquinol_10_ameliorates_mitochondrial_encephalopathy_associated_with_CoQ_deficiency_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0925-4439(14)00047-7 DB - PRIME DP - Unbound Medicine ER -