Coenzyme Q10 Analogues: Benefits and Challenges for Therapeutics

Suárez-Rivero JM, Pastor-Maldonado CJ, Povea-Cabello S, Álvarez-Córdoba M, Villalón-García I, Munuera-Cabeza M, Suárez-Carrillo A, Talaverón-Rey M, Sánchez-Alcázar JA.; Antioxidants. 2021; 10(2):236. doi:10.3390/antiox10020236

  Over the past few years, a wide variety of CoQ10 analogues with improved properties have been developed. These analogues conserve the antioxidant features of CoQ10 but present upgraded characteristics such as water solubility or enhanced mitochondrial accumulation. Moreover, recent studies have proven that some of these analogues might even outperform CoQ10 in the treatment of certain specific diseases. The aim of this review is to provide detailed information about these Coenzyme Q10 analogues, as well as their functionality and medical applications.

This synthetic quinone stands out for its efficacy as a treatment for Friedreich ataxia (FRDA). From 1990 and up to the present, several clinical trials have tested the impact of Idebenone supplementation on patients of FRDA. These studies have proven that the quinone ameliorates patients’ conditions through the improvement of neurological function (reduced general weakness, improvement in fine movement and speech, and decreased difficulty in swallowing) [49] and cardiac hypertrophy (reduction in interventricular septal wall thickness, left ventricular posterior wall thickness, or left ventricular mass index) [50,51]. Idebenone has been widely tested in clinical trials for several years. Its main relevance is in FRDA

Friedreich ataxia in a family from Mali, West Africa

heick Abdel Kader Cissé,Lassana Cissé,Hamidou O Bah,Oumar Samassékou,Assiatou Simaga,Abdoulaye Tamega,Salimata Diarra,Seybou Hassane Diallo,Thomas Coulibaly,Salimata Diallo,Abdoulaye Yalcouye,Alassane Baneye Maiga,Mohamed Kéita,Kenneth Fischbeck,Sekou Fantamady Traore,Cheick Guinto,Guida Landouré; West Africa. Authorea. February 24, 2021. DOI: 10.22541/au.161414432.28217723/v1 

Cissé, CAK, Cissé, L, Ba, HO, et al; from the H3Africa Consortium. Friedreich ataxia in a family from Mali, West Africa/Friedreich ataxia in a Malian family. Clin Case Rep. 2021; 9:e04065.  doi: 10.1002/ccr3.4065

 Friedreich ataxia is the most common inherited ataxia in the world, but yet to be reported in black African. We report the first genetically confirmed case in a West African family. Studying genetic diseases in populations with diverse backgrounds may give new insights into their pathophysiology for future therapeutic targets.

Crosstalk between nucleus and mitochondria in human disease: Mitochondrial iron and calcium homeostasis in Friedreich ataxia

Jordi Tamarit, Elena Britti, Fabien Delaspre, Marta Medina‐Carbonero, Arabela Sanz‐Alcázar, Elisa Cabiscol, Joaquim Ros; IUBMB Life. 2021; 1– 11. https://doi.org/10.1002/iub.2457

 Friedreich Ataxia is a neuro‐cardiodegenerative disease caused by the deficiency of frataxin, a mitochondrial protein. Many evidences indicate that frataxin deficiency causes an unbalance of iron homeostasis. Nevertheless, in the last decade many results also highlighted the importance of calcium unbalance in the deleterious downstream effects caused by frataxin deficiency. In this review, the role of these two metals has been gathered to give a whole view of how iron and calcium dyshomeostasys impacts on cellular functions and, as a result, which strategies can be followed to find an effective therapy for the disease.