A mitochondrial deficiency will result in elevated oxaloacetate:malate ratio.TrueFalse

The Krebs cycle still operates, even though the concentration of oxaloacetate in the mitochondrial matrix is low. Because:Group of answer choicesAcetyl-CoA is a negative allosteric modulator of the PDH complexthe hydrolysis of a thioester bond is highly favourableThe interconversion of L-malate and oxaloacetate is readily reversibleThe ΔG’0 for the formation of oxaloacetate from L-malate is large and positive

The activity of this enzyme is the main determinant of the rate of mitochondrial fatty acid oxidation:Group of answer choicesCPTPDHHAD

Medium-chain acyl-CoA dehydrogenase (MCAD) is an enzyme involved in β-oxidation and is encoded by the autosomal ACADM gene.  MCAD deficiency is caused by a mutation in ACADM and results in impaired ability to digest medium-chain fatty acids for energy and in their subsequent accumulation.  Which of the following interventions would best relieve the negative effects of MCAD deficiency?A.Mitochondrial gene therapyB.Supplementation with long-chain fatty acidsC.Prolonged periods of fastingD.A high-carbohydrate, low-fat diet

Recall the function of mitochondria.

Mitochondrial respiration was measured in isolated thoraces using high-resolution respirometry