کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8260171 | 1534658 | 2014 | 9 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency
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کلمات کلیدی
GFAPTuj18-OHdGUbiquinol-10ETFBN-PAGECoenzyme Q9CoQ9CoQ10CoQCoQ10 deficiencyH&E - H & EMitochondrial encephalopathy - آنسفالوپاتی میتوکندریElectron-transfer flavoprotein - فلو پروتئین انتقال الکترونMouse model - مدل موشHematoxylin and Eosin - هماتوکسیلین و ائوزین8-hydroxyguanosine - هیدروکسی گوانوزین 8glial fibrillary acid protein - پروتئین اسید فیبریلیری گلیالhigh-performance liquid chromatography - کروماتوگرافی مایعی کاراHPLC - کروماتوگرافی مایعی کاراCoenzyme Q - کوآنزیم QCoenzyme Q10 - کوآنزیم Q10
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
سالمندی
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چکیده انگلیسی
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 (Coq9X/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 Coq9X/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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1842, Issue 7, July 2014, Pages 893-901
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1842, Issue 7, July 2014, Pages 893-901
نویسندگان
Laura GarcÃa-Corzo, Marta Luna-Sánchez, Carolina Doerrier, Francisco Ortiz, Germaine Escames, DarÃo Acuña-Castroviejo, Luis C. López,