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Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders

Overview of attention for article published in Human Molecular Genetics, March 2014
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About this Attention Score

  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (88th percentile)
  • High Attention Score compared to outputs of the same age and source (85th percentile)

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1 blog
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2 X users
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1 patent
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1 Google+ user

Citations

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112 Dimensions

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185 Mendeley
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Title
Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders
Published in
Human Molecular Genetics, March 2014
DOI 10.1093/hmg/ddu123
Pubmed ID
Authors

Lindsay C. Burrage, Sandesh C.S. Nagamani, Philippe M. Campeau, Brendan H. Lee

Abstract

Branched-chain amino acid (BCAA) metabolism plays a central role in the pathophysiology of both rare inborn errors of metabolism and the more common multifactorial diseases. Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations in the BCAAs and their ketoacids have been recognized as the cause of maple syrup urine disease (MSUD) for decades, treatment options for this disorder have been limited to dietary interventions. In recent years, the discovery of improved leucine tolerance after liver transplantation has resulted in a new therapeutic strategy for this disorder. Likewise, targeting the regulation of the BCKDC activity may be an alternative potential treatment strategy for MSUD. The regulation of the BCKDC by the branched-chain ketoacid dehydrogenase kinase has also been implicated in a new inborn error of metabolism characterized by autism, intellectual disability and seizures. Finally, there is a growing body of literature implicating BCAA metabolism in more common disorders such as the metabolic syndrome, cancer and hepatic disease. This review surveys the knowledge acquired on the topic over the past 50 years and focuses on recent developments in the field of BCAA metabolism.

X Demographics

X Demographics

The data shown below were collected from the profiles of 2 X users who shared this research output. Click here to find out more about how the information was compiled.
Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 185 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United States 2 1%
Japan 1 <1%
Denmark 1 <1%
Unknown 181 98%

Demographic breakdown

Readers by professional status Count As %
Researcher 32 17%
Student > Bachelor 26 14%
Student > Ph. D. Student 21 11%
Student > Master 20 11%
Student > Postgraduate 11 6%
Other 30 16%
Unknown 45 24%
Readers by discipline Count As %
Medicine and Dentistry 37 20%
Agricultural and Biological Sciences 27 15%
Biochemistry, Genetics and Molecular Biology 22 12%
Nursing and Health Professions 15 8%
Pharmacology, Toxicology and Pharmaceutical Science 7 4%
Other 26 14%
Unknown 51 28%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 13. 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 15 December 2016.
All research outputs
#2,511,502
of 23,342,092 outputs
Outputs from Human Molecular Genetics
#811
of 8,070 outputs
Outputs of similar age
#26,373
of 224,707 outputs
Outputs of similar age from Human Molecular Genetics
#14
of 92 outputs
Altmetric has tracked 23,342,092 research outputs across all sources so far. Compared to these this one has done well and is in the 89th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 8,070 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.0. This one has done well, scoring higher than 89% of its peers.
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 224,707 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 88% of its contemporaries.
We're also able to compare this research output to 92 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 85% of its contemporaries.