Browsing by Subject "Ndufs4 knockout mice"
Now showing items 1-6 of 6
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Investigating tissue-specificity in a mouse model of Leigh Syndrome using metabolomics
(North-West University (South Africa), 2022)Mitochondrial disease (MD) is one of the most challenging groups of inborn disorders featuring a tissue-specific vulnerability, governed by largely unknown mechanisms. The value of metabolomics in multi-systemic MD ... -
Investigation into the protection of mitochondrial disease pathology by metallothionein overexpression
(North-West University (South Africa), 2020)Mitochondrial diseases (MD), such as Leigh Syndrome (LS), present with severe neurological and muscular phenotypes in patients, but have no known cure and limited treatment options. Based on their neuroprotective effects ... -
Metabolomics and biochemical evaluation of skeletal muscle from Ndufs4 knockout mice
(North-West University (South Africa), 2019)The dysfunction of mitochondrial complex I (CI) impedes the most efficient mechanism feeding electrons into the respiratory chain (RC) — a system that, together with ATP synthase (CV), is responsible for the majority of ... -
Metallothionein 1 overexpression does not protect against mitochondrial disease pathology in Ndufs4 knockout mice
(Springer, 2021)Mitochondrial diseases (MD), such as Leigh syndrome (LS), present with severe neurological and muscular phenotypes in patients, but have no known cure and limited treatment options. Based on their neuroprotective effects ... -
Sub-cellular metabolomics investigation of mitochondrial CI deficiency in cytosol & mitochondria of Ndufs4 mice
(North-West University (South Africa), 2022)Mitochondrial Complex I (CI) is regarded as the foremost entry point for electrons that fuel oxidative phosphorylation. CI deficiency (CID) is the most common mitochondrial disease (MD) in humans and Leigh syndrome (LS), ... -
Urinary metabolomics investigation of Ndufs4 knockout mice
(North-West University (South Africa), 2020)Mitochondrial diseases (MDs) are the most common inborn errors of metabolism, with an estimated prevalence of approximately 1 in 5 000 live births, and are mainly caused by deficiencies of complex I (CI) of the oxidative ...
