Fatty acid metabolism in dystrophic muscle

Lin, C.H.; Hudson, Arthur J.; Strickland, K. P.

doi:10.1016/0024-3205(69)90112-x

Cited by 40 publications

(16 citation statements)

References 9 publications

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“…he findings in Table 1 with dystrophic hamOxidative phosphorylation parameters were also mea-ster mitochondria confirmed the depressed mte sured polarographically (Qxygraph, G.M.E., Madison) as described previously (7) but using a Clarke electrode at of palmit ate utilization described by St rickland -0.8 V. The reaction mediuan, saturated with air at 28 "C, and his co-workers in dystrophic inice (4,5). With ADP to deplete the endogenous substrate.…”

Section: Resultssupporting

confidence: 83%

“…However, mitochondria prepared without Nagarse oxidized palmitate at a rate comparable to that reported by Lin et al (4). Since the 8, uptake measured manometrically and the 14c0, production both exhibited a sigmoid curve with time, it was more convenient to estimate the rate from the linear portion of the 0, curve rather than from multiple I4C8, determinations.…”

Section: Resultssupporting

confidence: 69%

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Defective respiration and oxidative phosphorylation in muscle mitochondria of hamsters in the late stages of hereditary muscular dystrophy

Jacobson¹,

Blanchaer²,

Wrogemann³

1970

Can. J. Biochem.

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Defective respiration and oxidative phosphorylation in muscle mitochondria of hamsters in the late stages of hereditary muscular dystrophy. Can. J. Biochem. 48, 1037Biochem. 48, -1042Biochem. 48, (1970.Skeletal muscle mitochondria were isolated from 33 dystrophic hamsters of the BI8 14.6 strain, aged 265 f 13 (S.E.) days, by glass-on-glass homogenization in a sucrose-EDTA medium in the absence of the proteinase Nagarse. These organelles utilized O2 at half the normal rate with pyruvatelfumarate or palmitate as substrate in a manometric test system and exhibited decreased P/Q ratios and phospkorylation rates with pyruvateifumarate. In pslarographic experiments the mitochondria from dystrophic muscle, supplemented with L-malate, had significantly depressed O2 uptake rates, respiratory control ratios, and phosphorylation rates with pyruvate, palmityl-L-carnitine, and acetyl-L-carnitine as substrates and low ADP/O ratios with pyruvate and palmityl-~~carnitine. Since the severity of the respiratory depression was similar with the three substrates, it appeared that the defect lay beyond acetyl-CoA in their common degradative pathway. Judging from the rapid rate of succinate and NABH oxidation, the respiratory chain was unimpaired. It was concluded that a defect was present in the tricarboxylic acid cycle of muscle mitochondria isolated without Nagarse from older dystrophic hamsters of the BZO 14.6 strain and that the defect was accompanied by a loose coupling of oxidative phosphorylation.

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Section: Resultssupporting

confidence: 83%

Section: Resultssupporting

confidence: 69%

Defective respiration and oxidative phosphorylation in muscle mitochondria of hamsters in the late stages of hereditary muscular dystrophy

Jacobson¹,

Blanchaer²,

Wrogemann³

1970

Can. J. Biochem.

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“…Recent studies have demonstrated that in the skeletal muscle of chickens with hereditary muscular dystrophy several metabolic processes directly affected by cyclic AMP are significantly altered during maturation compared to normal animals [ [9,13]. It has also been reported that the skeletal muscle of mice afflicted with muscular dystrophy contains elevated levels of cyclic AMP [8].…”

Section: Introductionmentioning

confidence: 99%

Adenyl Cyclase in the Pectoral Mnscle of Normal Chickens and Chickens with Hereditary Muscular Dystrophy

Bz¹,

Dh²

1972

Enzyme

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Adenyl cyclase activity was determined in the pectoral muscle of normal chickens and chickens with hereditary muscular dystrophy at various stages of development. Significant differences were found in enzyme activity between these two types of tissue. The muscle of both normal and dystrophic embryos contained high levels of enzyme activity which decreased shortly after hatching. By 12 weeks ex ovo enzyme titers in the normal pectoralis were sharply reduced and remained unchanged up to 60 weeks. By contrast, enzyme levels in the dystrophic pectoralis declined to a lesser degree after hatching and then rose sharply beginning at 12 weeks ex ovo and remained high in the mature bird. Cyclic AMP phosphodiesterase activity levels were altered in a similar manner in the two tissues.

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“…The results obtained on the activities of the citric acid cycle and respiratory chain enzymes indicate that the impairment in the utilization of acetyl-1-carnitine [3], pyruvate and palmitic acid [6] occurring in the isolated muscle mitochondria from dystrophic mice is not due to an enzymatic deficiency in either of these systems. However, these results do not rule out the possibility of an alteration in the overall function of the cycle.…”

Section: Discussionmentioning

confidence: 86%

“…Also, a de crease in the oxidation of palmitate-l-14C and pyruvate-14C has been previ ously reported [6]. These findings suggested an alteration in the operation of the citric acid cycle and, therefore, a study was made of the activities of individual enzymes in the citric acid cycle and also the electron transport chain of dystrophic mouse skeletal muscle.…”

Section: Introductionmentioning

confidence: 88%

Activities of Enzymes of the Citric Acid Cycle and Electron Transport Chain in the Skeletal Muscle of Normal and Dystrophic Mice (Strain 129)

Jj¹,

Aj²,

Kp³

1972

Enzyme

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The enzymatic activities of the citric acid cycle and the electron transport chain have been determined in mitochondria isolated from the hind leg muscle of normal and dystrophic mice (strain 129). No significant difference between enzymatic activity of normal and dystrophic muscle was found with any of the enzymes studied. There is evidence of an increased mitochondrial content in dystrophic muscle as calculated by succinate dehydrogenase activity in whole muscle homogenate relative to the activity in isolated mitochondria. Despite the increased mitochondrial content there is a lower yield of mitochondria from dystrophic muscle that is believed due to increased fragility of the mitochondria in this condition.

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Fatty acid metabolism in dystrophic muscle

Cited by 40 publications

References 9 publications

Defective respiration and oxidative phosphorylation in muscle mitochondria of hamsters in the late stages of hereditary muscular dystrophy

Defective respiration and oxidative phosphorylation in muscle mitochondria of hamsters in the late stages of hereditary muscular dystrophy

Adenyl Cyclase in the Pectoral Mnscle of Normal Chickens and Chickens with Hereditary Muscular Dystrophy

Activities of Enzymes of the Citric Acid Cycle and Electron Transport Chain in the Skeletal Muscle of Normal and Dystrophic Mice (Strain 129)

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