La chaîne de transport d'électrons: La chaîne respiratoire est composée de cinq gradient de pH entre la matrice mitochondriale et l'espace. The Full Text of this article is available as a PDF (K). enzymatiques de la chaîne respiratoire mitochondriale dans les myocardiopathies de l'enfant. Les principales fonctions des mitochondries. Les mitochondries, au travers de l' activité de la chaîne respiratoire (composée des complexes I à. 1: Mécanismes de transport des protons par la chaîne respiratoire (F. Thème A . L'oxydation du NADH par les mitochondries isolées. sub-cellular level of mitochondrial function, was that introgressed S. La majorité des enzymes impliquées dans la phosphorylation oxydative (chaîne de thermique des enzymes respiratoires mitochondriales codées par l'ADNmt et d' une. There is growing evidence that mitochondrial dysfunction, and more specifically (ERO) par la chaîne respiratoire endommagée. En effet.
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Previous Searches. My Records. Brief view. Table view. Full view. Sort by:. Title Creator Subject Faculty Date. Record 1 of 1. Mitochondrial function in cells, tissues and animals without ubiquinone biosynthesis.
Wang, Ying. Ubiquinone Coenzyme Q, UQ or CoQ is a small lipophilic molecule which is essential for all life forms that rely on mitochondrial respiration for energy production.
It functions as an electron carrier in the respiratory chain and plays a role in many other cellular functions as well. In the past decade, an increasing number of patients have been described to have a genetic defect in UQ biosynthesis and present with severe and diverse clinical manifestations, making UQ research relevant to patient care.
I generated three conditional Mclk1 knockout KO models which la chaine respiratoire mitochondriale pdf able to bypass the embryonic lethality of Mclk1 and allow us to examine the consequences of complete loss-of-function of Mclk1 in vitro and in vivo in adult animals.
They are viable under standard glucose culture conditions. More interestingly, despite lacking UQ, they still have a functionally active electron transport chain. These observations suggest that 1 the respiratory phenotype of Mclk1 mutants can be considered essentially as a UQ-deficiency phenotype, and 2 mitochondrial respiration can occur in the absence of UQ. La chaine respiratoire mitochondriale pdf KO MEFs cannot survive in medium containing galactose instead of glucose la chaine respiratoire mitochondriale pdf this forces cells to mostly rely on mitochondrial ATP production to sustain their viability.
These characteristics make them a unique tool for testing the efficacy of UQ analogues in la chaine respiratoire mitochondriale pdf electron transport in mammalian mitochondria. Next, we generated a mouse model with liver-specific loss of Mclk1. A large depletion of UQ in hepatocytes was found to cause only a mild impairment of respiratory chain function and no gross abnormalities.
Therefore, liver mitochondrial function appears to have a high tolerance of severe UQ deficiency. Using this model, we also demonstrated that dietary UQ10 can functionally rescue the endogenous UQ deficiency of Mclk1 KO liver at the respiratory chain level. Furthermore, we generated an inducible Mclk1 KO model in which Mclk1 was knocked out in adult mice.
These animals have a median survival time of 9 months after induction of global KO of la chaine respiratoire mitochondriale pdf Mclk1 gene. We observed a very slow gradual decrement of tissue UQ contents after inducing the ablation of Mclk1, which could explain the slowly progressive phenotype in these mice.
In the heart, kidneys and skeletal muscles severe deficits of UQ were found to severely impair mitochondrial respiration. Therefore, unlike the liver, the kidneys and muscle tissues need high levels of UQ najee tokyo blue sustain sufficient respiratory function.
I examined the efficacy of dietary UQ10 supplementation against the global KO model and found that dietary UQ10 cannot be taken up by other organs beside the liver, and accordingly it was almost completely ineffective in rescuing the Mclk1 KO phenotype.
On the other hand, feeding the mutant animals with 2,4-dihydroxybenzoic acid, a synthetic analogue of the UQ ring precursor 4-hydroxybenzoic acid 4-HBled to dramatic rescue of disease phenotypes in these animals, including their early lethality. This finding suggests that some types of primary UQ deficiency could be relieved by treatment with the appropriate unnatural precursors, and more generally, that biosynthetic "by-pass" precursors could be a new treatment ddlx evo 5 for biosynthetic pathway defects.
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