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Human induced pluripotent stem cell derived neurons as a model for Williams-Beuren syndrome

Overview of attention for article published in Molecular Brain, November 2015
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  • Good Attention Score compared to outputs of the same age (75th percentile)
  • Above-average Attention Score compared to outputs of the same age and source (52nd percentile)

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8 X users

Citations

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35 Dimensions

Readers on

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73 Mendeley
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Title
Human induced pluripotent stem cell derived neurons as a model for Williams-Beuren syndrome
Published in
Molecular Brain, November 2015
DOI 10.1186/s13041-015-0168-0
Pubmed ID
Authors

Shahryar Khattak, Elise Brimble, Wenbo Zhang, Kirill Zaslavsky, Emma Strong, P. Joel Ross, Jason Hendry, Seema Mital, Michael W. Salter, Lucy R. Osborne, James Ellis

Abstract

Williams-Beuren Syndrome (WBS) is caused by the microdeletion of approximately 25 genes on chromosome 7q11.23, and is characterized by a spectrum of cognitive and behavioural features. We generated cortical neurons from a WBS individual and unaffected (WT) control by directed differentiation of induced pluripotent stem cells (iPSCs). Single cell mRNA analyses and immunostaining demonstrated very efficient production of differentiated cells expressing markers of mature neurons of mixed subtypes and from multiple cortical layers. We found that there was a profound alteration in action potentials, with significantly prolonged WBS repolarization times and a WBS deficit in voltage-activated K(+) currents. Miniature excitatory synaptic currents were normal, indicating that unitary excitatory synaptic transmission was not altered. Gene expression profiling identified 136 negatively enriched gene sets in WBS compared to WT neurons including gene sets involved in neurotransmitter receptor activity, synaptic assembly, and potassium channel complexes. Our findings provide insight into gene dysregulation and electrophysiological defects in WBS patient neurons.

X Demographics

X Demographics

The data shown below were collected from the profiles of 8 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 73 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Japan 1 1%
Unknown 72 99%

Demographic breakdown

Readers by professional status Count As %
Student > Master 16 22%
Student > Ph. D. Student 14 19%
Researcher 13 18%
Student > Bachelor 6 8%
Other 4 5%
Other 8 11%
Unknown 12 16%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 20 27%
Agricultural and Biological Sciences 14 19%
Medicine and Dentistry 8 11%
Neuroscience 7 10%
Pharmacology, Toxicology and Pharmaceutical Science 2 3%
Other 5 7%
Unknown 17 23%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 5. 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 22 September 2016.
All research outputs
#6,875,065
of 25,373,627 outputs
Outputs from Molecular Brain
#307
of 1,198 outputs
Outputs of similar age
#96,736
of 392,980 outputs
Outputs of similar age from Molecular Brain
#16
of 34 outputs
Altmetric has tracked 25,373,627 research outputs across all sources so far. This one has received more attention than most of these and is in the 72nd percentile.
So far Altmetric has tracked 1,198 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.9. This one has gotten more attention than average, scoring higher than 74% 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 392,980 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 75% of its contemporaries.
We're also able to compare this research output to 34 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 52% of its contemporaries.