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

Overview of attention for article published in Pediatric Research, December 1992
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About this Attention Score

  • Above-average Attention Score compared to outputs of the same age (60th percentile)
  • Above-average Attention Score compared to outputs of the same age and source (53rd percentile)

Mentioned by

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1 Wikipedia page

Citations

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59 Dimensions

Readers on

mendeley
13 Mendeley
Title
Mitochondrial ATP-Synthase Deficiency in a Child with 3-Methylglutaconic Aciduria
Published in
Pediatric Research, December 1992
DOI 10.1203/00006450-199212000-00022
Pubmed ID
Authors

Elisabeth Holme, Joachim Greter, Carl-Eric Jacobson, Nils-Göran Larsson, Sven Lindstedt, Karl Olof Nilsson, Anders Oldfors, Már Tulinius

Abstract

We report the finding of mitochondrial ATP-synthase 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-HCO3 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-1 x (mg protein)-1, control range 223-673 nmol x min-1 x (mg protein)-1. The mitochondrial respiratory rate was low and tightly coupled. The respiratory rate was normalized by the addition of an uncoupler. Low Mg2+ 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.

Mendeley readers

The data shown below were compiled from readership statistics for 13 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 13 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 3 23%
Student > Master 2 15%
Student > Ph. D. Student 2 15%
Other 1 8%
Professor 1 8%
Other 2 15%
Unknown 2 15%
Readers by discipline Count As %
Agricultural and Biological Sciences 3 23%
Biochemistry, Genetics and Molecular Biology 2 15%
Psychology 1 8%
Social Sciences 1 8%
Neuroscience 1 8%
Other 0 0%
Unknown 5 38%

Attention Score in Context

This research output has an Altmetric Attention Score of 3. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 24 August 2018.
All research outputs
#3,994,809
of 13,415,596 outputs
Outputs from Pediatric Research
#864
of 3,108 outputs
Outputs of similar age
#102,621
of 267,821 outputs
Outputs of similar age from Pediatric Research
#37
of 79 outputs
Altmetric has tracked 13,415,596 research outputs across all sources so far. This one is in the 49th percentile – i.e., 49% of other outputs scored the same or lower than it.
So far Altmetric has tracked 3,108 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.1. This one is in the 41st percentile – i.e., 41% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 267,821 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 60% of its contemporaries.
We're also able to compare this research output to 79 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 53% of its contemporaries.