The Experts below are selected from a list of 36 Experts worldwide ranked by ideXlab platform

Duarte J. - One of the best experts on this subject based on the ideXlab platform.

  • Beta-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9(R239X) mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9(R239X) mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Beta-Resorcylic Acid (Beta-RA), a structural analog of the CoQ precursor 4-hydroxybenzoic Acid and the anti-inflammatory salicylic Acid. Beta-RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone-9 (DMQ(9)) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down-regulation of astrocytes-related neuroinflammatory genes. Because the therapeutic outcomes of Beta-RA administration were superior to those after CoQ(10) supplementation, its use in the clinic should be considered in CoQ deficiencies

  • Β‐RA reduced DMQ/CoQ ration and rescues the encephalopathic phenotype in Coq9R239X mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9R239X mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Β‐Resorcylic Acid (Β‐RA), a structural analog of the CoQ precursor 4‐hydroxybenzoic Acid and the anti‐inflammatory salicylic Acid. Β‐RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone‐9 (DMQ9) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down‐regulation of astrocytes‐related neuroinflammatory genes. Because the therapeutic outcomes of Β‐RA administration were superior to those after CoQ10 supplementation, its use in the clinic should be considered in CoQ deficiencies

Hidalgo-gutiérrez A. - One of the best experts on this subject based on the ideXlab platform.

  • Beta-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9(R239X) mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9(R239X) mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Beta-Resorcylic Acid (Beta-RA), a structural analog of the CoQ precursor 4-hydroxybenzoic Acid and the anti-inflammatory salicylic Acid. Beta-RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone-9 (DMQ(9)) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down-regulation of astrocytes-related neuroinflammatory genes. Because the therapeutic outcomes of Beta-RA administration were superior to those after CoQ(10) supplementation, its use in the clinic should be considered in CoQ deficiencies

  • Β‐RA reduced DMQ/CoQ ration and rescues the encephalopathic phenotype in Coq9R239X mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9R239X mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Β‐Resorcylic Acid (Β‐RA), a structural analog of the CoQ precursor 4‐hydroxybenzoic Acid and the anti‐inflammatory salicylic Acid. Β‐RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone‐9 (DMQ9) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down‐regulation of astrocytes‐related neuroinflammatory genes. Because the therapeutic outcomes of Β‐RA administration were superior to those after CoQ10 supplementation, its use in the clinic should be considered in CoQ deficiencies

Lioubov Kiwi-minsker - One of the best experts on this subject based on the ideXlab platform.

  • Intensification of slow reversible chemical transformation: carboxylation of resorcinol as a case study
    Green Processing and Synthesis, 2013
    Co-Authors: Charline Berguerand, Anne-laure Dessimoz, Lioubov Kiwi-minsker
    Abstract:

    Conjugated kinetic and thermodynamic modeling is suggested as a suitable approach to identify a novel process window (NPW) for the intensification of slow reversible reactions. The aqueous Kolbe-Schmitt synthesis of Beta-Resorcylic Acid is taken as a model. The potential of operating at high pressure (P) and temperature (T) is evaluated in order to reduce the characteristic reaction time (t(r)) and increase the specific productivity. For the first time, a reliable kinetic model for this reaction is derived from batch experiments. Based on this model, an NPW of P =10 bars, T=453 K is determined for a continuous reactor operated at a residence time of 28 s. It is predicted that the specific productivity can be increased by a factor of 100 with a 4.2 times less concentrated KHCO3 solution, as compared to a conventional batch process, if the reaction is kinetically controlled. The model prediction is experimentally validated with a continuously operated milli-reactor equipped by SMXS mixer elements (Sulzer Chemtech, Switzerland) ensuring a fast mixing (characteristic mixing time similar to 4.10(-2) s). The milli-reactor renders exclusively Beta-Resorcylic Acid (selectivity 100%) and confirms process intensification (PI) of two orders of magnitude as compared to conventional batch operation.

  • Kinetic and thermodynamic study of the aqueous Kolbe–Schmitt synthesis of Beta-Resorcylic Acid
    Chemical Engineering Journal, 2012
    Co-Authors: Anne-laure Dessimoz, Albert Renken, Charline Berguerand, Lioubov Kiwi-minsker
    Abstract:

    Detailed thermodynamic and kinetic study of the aqueous Kolbe-Schmitt synthesis of Beta-Resorcylic Acid has been carried out in a laboratory-scale batch reactor operated under ambient pressure. The dependence of the conversion on partial pressure of carbon dioxide (4 x 10(-4) - 1 bar), initial concentration of resorcinol (0.4-0.8 M), initial concentration of potassium bicarbonate (0.4-6 M) and temperature (348-428 K) was studied. The reaction was found to be reversible and exothermic (Delta H = -30.4 kJ/mol). Partial pressure of carbon dioxide does not influence the reaction kinetics when carried out in the excess of potassium bicarbonate (C-KHCO3,C-0/C-R,C-0 > 1). Increase of the initial concentration of potassium bicarbonate accelerates drastically the rate and shifts the equilibrium conversion to higher values. For the first time a reliable kinetic model has been proposed based on two reversible reactions of pseudo first order towards resorcinol and Beta-Resorcylic Acid. The kinetic constants showed Arrhenius dependencies on the reaction temperature with the apparent energies of activation 105 and 125 kJ/mol for the forward and the reversed reactions, respectively. Pre-exponential factors increased with the initial concentration of potassium bicarbonate indicating a complexity of the reaction mechanism involved. The kinetic model was successfully validated against experimental data obtained under high pressure and temperature using a micro-plant. Finally, the model was found consistent with the literature data available for this reaction. (C) 2012 Elsevier B.V. All rights reserved.

Bakkali M. - One of the best experts on this subject based on the ideXlab platform.

  • Beta-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9(R239X) mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9(R239X) mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Beta-Resorcylic Acid (Beta-RA), a structural analog of the CoQ precursor 4-hydroxybenzoic Acid and the anti-inflammatory salicylic Acid. Beta-RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone-9 (DMQ(9)) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down-regulation of astrocytes-related neuroinflammatory genes. Because the therapeutic outcomes of Beta-RA administration were superior to those after CoQ(10) supplementation, its use in the clinic should be considered in CoQ deficiencies

  • Β‐RA reduced DMQ/CoQ ration and rescues the encephalopathic phenotype in Coq9R239X mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9R239X mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Β‐Resorcylic Acid (Β‐RA), a structural analog of the CoQ precursor 4‐hydroxybenzoic Acid and the anti‐inflammatory salicylic Acid. Β‐RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone‐9 (DMQ9) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down‐regulation of astrocytes‐related neuroinflammatory genes. Because the therapeutic outcomes of Β‐RA administration were superior to those after CoQ10 supplementation, its use in the clinic should be considered in CoQ deficiencies

Escames G. - One of the best experts on this subject based on the ideXlab platform.

  • Beta-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9(R239X) mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9(R239X) mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Beta-Resorcylic Acid (Beta-RA), a structural analog of the CoQ precursor 4-hydroxybenzoic Acid and the anti-inflammatory salicylic Acid. Beta-RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone-9 (DMQ(9)) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down-regulation of astrocytes-related neuroinflammatory genes. Because the therapeutic outcomes of Beta-RA administration were superior to those after CoQ(10) supplementation, its use in the clinic should be considered in CoQ deficiencies

  • Β‐RA reduced DMQ/CoQ ration and rescues the encephalopathic phenotype in Coq9R239X mice.
    'EMBO', 2018
    Co-Authors: Hidalgo-gutiérrez A., Barriocanal-casado E., Bakkali M., Díaz-casado M.e., Sánchez-maldonado L., Romero M., Sayed R.k., Prehn C., Escames G., Duarte J.
    Abstract:

    Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9R239X mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of Β‐Resorcylic Acid (Β‐RA), a structural analog of the CoQ precursor 4‐hydroxybenzoic Acid and the anti‐inflammatory salicylic Acid. Β‐RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone‐9 (DMQ9) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down‐regulation of astrocytes‐related neuroinflammatory genes. Because the therapeutic outcomes of Β‐RA administration were superior to those after CoQ10 supplementation, its use in the clinic should be considered in CoQ deficiencies