Respiratory Chain Defects May Present Only with Hypoglycemia

Defects in multiple complexes of the respiratory chain are present in ageing human colonic crypts

Mitochondrial DNA (mtDNA) mutations accumulate in a number of ageing tissues and are proposed to play a role in the ageing process. We have previously shown that colonic crypt stem cells accumulate somatic mtDNA point mutations during ageing. These mtDNA mutations result in the loss of the activity of complex IV (cytochrome c oxidase (COX)) of the respiratory chain in the stem cells and their p...

Respiratory chain enzyme defects in patients with idiopathic inflammatory myopathy.

OBJECTIVE To analyse muscle respiratory chain enzymes in idiopathic inflammatory myopathy. METHODS Four consecutive female patients seen at our hospital with idiopathic inflammatory myopathy were studied. Muscle histochemical staining included NADH tetrazolium reductase and succinate dehydrogenase tests. Activity of rotenone sensitive NADH cytochrome c reductase (complex I and III) succinate ...

Inborn and induced defects of the mitochondrial respiratory chain.

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Fumarate Hydratase Loss Causes Combined Respiratory Chain Defects

Fumarate hydratase (FH) is an enzyme of the tricarboxylic acid (TCA) cycle mutated in hereditary and sporadic cancers. Despite recent advances in understanding its role in tumorigenesis, the effects of FH loss on mitochondrial metabolism are still unclear. Here, we used mouse and human cell lines to assess mitochondrial function of FH-deficient cells. We found that human and mouse FH-deficient ...

Insulin Therapy and Hypoglycemia - Present and Future