2008
DOI: 10.1042/bsr20080090
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The mitochondrial pool of free amino acids reflects the composition of mitochondrial DNA-encoded proteins: indication of a post- translational quality control for protein synthesis

Abstract: Mitochondria can synthesize a limited number of proteins encoded by mtDNA by using its own biosynthetic machinery, whereas most of the proteins are imported from the cytosol. It could be hypothesized that the mitochondrial pool of amino acids follows the frequency of amino acids in mtDNA-encoded proteins or alternatively, the profile is the result of the participation of amino acids in pathways other than protein synthesis (e.g. heme biosynthesis, aminotransferase reactions). These hypotheses were tested by ev… Show more

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Cited by 27 publications
(28 citation statements)
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“…The column was calibrated using a calibration kit (Sigma). For comparison of the rates of transfer to ISCU and RSH, a first-order rate constant for sulfur transfer to RSH has been computed assuming a concentration of 100 mM of L-cysteine that is close to the physiological concentration 50 .…”
Section: Methodsmentioning
confidence: 99%
“…The column was calibrated using a calibration kit (Sigma). For comparison of the rates of transfer to ISCU and RSH, a first-order rate constant for sulfur transfer to RSH has been computed assuming a concentration of 100 mM of L-cysteine that is close to the physiological concentration 50 .…”
Section: Methodsmentioning
confidence: 99%
“…The Thr-deficient diets provided other essential amino acids at ratios based on egg albumin (Table 1; Ref. 45). Thr was set at 0, 30, 60, or 140% of the reported growth requirement in the 0.00, 0.18, 0.36, and 0.88% Thr diets, respectively (Table 2; Ref.…”
Section: Methodsmentioning
confidence: 99%
“…It has been proposed that variations in expression of mitochondrial genes in striated muscle are determined predominantly by gene dosage, rather than by modulation of transcriptional efficiency (74,(92)(93)(94). In some cases, overreplication of mtDNA aids with the normalization of mRNA levels of deleted genes as well as those nondeleted (74).…”
Section: Higher Mitochondrial Calcium In Ryr1 R163c Skeletal Muscle Imentioning
confidence: 99%