Browsing by Subject "Complex I deficiency"
Now showing items 1-8 of 8
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The effect of metallothionein overexpression on the transcription of selected genes involved in one-carbon metabolism and oxidative stress in NDUFS4-deficient mouse tissues
(North-West University (South Africa), 2020)Respiratory complex I (CI) defects are the most common mitochondrial disease, for which treatment is limited. Additionally, there is a lack of understanding of the impact of a CI defect upon the gene expression regulation ... -
Evaluation of metallothionein involvement in the modulation of mitochondrial respiration in mice
(North-West University, 2011)Metallothioneins (MTs) are small, non-enzymatic proteins that are involved in cellular detoxification and metal homeostasis because of their high cysteine content. MTs have also been identified as one of the vast number ... -
Investigating the adaptive mitochondrial shuttles and metabolic reprogramming of transporters in complex I (Ndufs4) knockout mice
(North-West University (South Africa), 2022)Mitochondrial disease is one of the most prevalent inherited paediatric disorders among children, with a prevalence of 1 in 5000 children. Leigh syndrome is the result of a Complex I (CI) deficiency and disrupts the redox ... -
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 ... -
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 ... -
A metabolomics and biochemical investigation of selected brain regions from Ndufs4 knockout mice
(North-West University (South Africa)North-West University, 2020)Mitochondria, the organelles found throughout the cytoplasm of most eukaryotic cells, have essential functions which have been implicated in the etiology of numerous metabolic and degenerative diseases. The mitochondrial ... -
Metabolomics of Ndufs4−/− skeletal muscle: adaptive mechanisms converge at the ubiquinone-cycle
(Elsevier, 2019)Leigh syndrome is one of the most common childhood-onset neurometabolic disorders resulting from a primary oxidative phosphorylation dysfunction and affecting mostly brain tissues. Ndufs4−/− mice have been widely used to ... -
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 ...
