The histochemical characterization of the coupling state of skeletal muscle mitochondria

Meijer, A. E. F. H.; Vloedman, A. H. T.

doi:10.1007/bf00489769

Cited by 45 publications

(19 citation statements)

References 57 publications

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“…Morphologic examination, including enzyme histochemical and ultrastructural analysis of muscle tissue, was performed as described (10). Enzyme histochemical staining for mitochondrial Mg2+ ATPase was performed as described (27). Biochemical investigation of the respiratory chain function was done on isolated intact muscle mitochondria with polarographic measurements as described (10,28) and in disrupted isolated mitochondria by spectrophotometric methods as described (1 0).…”

Section: Methodsmentioning

confidence: 99%

Mitochondrial ATP-Synthase Deficiency in a Child with 3-Methylglutaconic Aciduria

et al. 1992

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ABSTRACT. We report the finding of mitochondrial ATPsynthase deficiency in a child with persistent 3-methylglutaconic aciduria. The child presented in the neonatal period with severe lactic acidosis, which was controlled by Na-H C 0 3 and glucose infusions. During the 1st y of life, there were several episodes of lactic acidosis precipitated by infections or prolonged intervals between meals. The excretion of lactate in urine was variable, but there was a persistent high excretion of 3-methylglutaconic acid. The activity of 3-methylglutaconyl-CoA hydratase in fibroblasts was normal. The child had a hypertrophic cardiomyopathy and magnetic resonance images revealed hypoplasia of corpus callosum. The gross motor and mental development was retarded, but there were no other neurologic signs. Investigation of muscle mitochondrial function at 1 y of age revealed a severe mitochondrial ATP-synthase deficiency (oligomycin-sensitive, dinitrophenol-stimulated Mg2+ ATPase activity: 27 nmol x min-' x (mg protein)-', control range 223-673 nmol x min-' x (mg protein)-'.The mitochondrial respiratory rate was low and tightly coupled. The respiratory rate was normalized by the addition of an uncoupler. Low MgZ+ ATPase activity was also demonstrated by histochemical methods. Morphologic examination revealed ultrastructural abnormalities of mitochondria. There was no deletion of mitochondrial DNA. The sequences of the ATP synthase subunit genes of mitochondrial DNA were in accordance with published normal sequences. (Pediatr Res 32: 731-735,1992)

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Section: Methodsmentioning

confidence: 99%

Mitochondrial ATP-Synthase Deficiency in a Child with 3-Methylglutaconic Aciduria

et al. 1992

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“…The method is a modification of that published by Rosenblatt et al (1987), which is in tum a combination of two previously reported techniques (Meijer and Vloedman, 1980;Meijer, 1970). The histochemical bases of the staining reaction are those reported by Meijer (1970) and Meijer and Vossenberg (1977) and quoted by Rosenblatt et al (1987).…”

Section: Discussionmentioning

confidence: 99%

A histochemical ATPase method for the demonstration of the muscle capillary network.

Fouces

Torrella²,

Palomeque³

et al. 1993

J Histochem Cytochem.

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A histochemical method for demonstration of the cap== in skeletal muscle of birds is proposed. The present method, which is a modification of a previously reported myosin ATPase technique used for simultaneous staining of capillaries and fiber types, provides an accurate count of capillaries associated with different fiber types in avian skeletal musdes. We have applied the original and the modified method to serial adjacent sections of certain skeletal musdes and our results show that after the application of the original technique: (a) in muscles having dark Type Ii fibers, these fibers produce a masking effect on their adjacent capillaries: (b) a consistent and significant undetcounting in cap-

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“…This staining is weak in basal conditions but increases under the action of dinitrophenot (uncoupler) Meijer and Vloedman 1980).…”

Section: Histoenzymologymentioning

confidence: 99%

Morphological studies of skeletal muscle in lactic acidosis

Romero

Lombès

Touati

et al. 1996

J of Inher Metab Disea

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This paper underscores the contribution of routine morphological examination of skeletal muscle in patients with lactic acidosis. Mitochondrial disorders are by far the most common causes of primary lactic acidosis, in which muscle biopsy analysis helps in diagnosis and in the search for the molecular anomalies. Thus, we focus our attention on one particular point: the contribution of the morphological study of muscle biopsy in primary lactic acidosis due to mitochondrial disorders, especially mitochondrial respiratory-chain diseases.

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The histochemical characterization of the coupling state of skeletal muscle mitochondria

Cited by 45 publications

References 57 publications

Mitochondrial ATP-Synthase Deficiency in a Child with 3-Methylglutaconic Aciduria

Mitochondrial ATP-Synthase Deficiency in a Child with 3-Methylglutaconic Aciduria

A histochemical ATPase method for the demonstration of the muscle capillary network.

Morphological studies of skeletal muscle in lactic acidosis

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