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Generation and Neuronal Differentiation of hiPSCs From Patients With Myotonic Dystrophy Type 2

Overview of attention for article published in Frontiers in Physiology, July 2018
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Title
Generation and Neuronal Differentiation of hiPSCs From Patients With Myotonic Dystrophy Type 2
Published in
Frontiers in Physiology, July 2018
DOI 10.3389/fphys.2018.00967
Pubmed ID
Authors

Paola Spitalieri, Rosa V. Talarico, Michela Murdocca, Luana Fontana, Marzia Marcaurelio, Elena Campione, Roberto Massa, Giovanni Meola, Annalucia Serafino, Giuseppe Novelli, Federica Sangiuolo, Annalisa Botta

Abstract

Human induced pluripotent stem cells (hiPSCs)-patient specific are an innovative tool to reproduce a model of disease in vitro and summarize the pathological phenotype and the disease etiopathology. Myotonic dystrophy type 2 (DM2) is caused by an unstable (CCTG)n expansion in intron 1 of the CNBP gene, leading to a progressive multisystemic disease with muscle, heart and central nervous dysfunctions. The pathogenesis of CNS involvement in DM2 is poorly understood since no cellular or animal models fully recapitulate the molecular and clinical neurodegenerative phenotype of patients. In this study, we generated for the first time, two DM2 and two wild type hiPSC lines from dermal fibroblasts by polycistronic lentiviral vector (hSTEMCCA-loxP) expressing OCT4, SOX2, KLF4, and cMYC genes and containing loxP-sites, excisable by Cre recombinase. Specific morphological, molecular and immunocytochemical markers have confirmed the stemness of DM2 and wild type-derived hiPSCs. These cells are able to differentiate into neuronal population (NP) expressing tissue specific markers. hiPSCs-derived NP cells maintain (CCTG)n repeat expansion and intranuclear RNA foci exhibiting sequestration of MBNL1 protein, which are pathognomonic of the disease. DM2 hiPSCs represent an important tool for the study of CNS pathogenesis in patients, opening new perspectives for the development of cell-based therapies in the field of personalized medicine and drug screening.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 19 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 19 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 3 16%
Student > Doctoral Student 3 16%
Student > Bachelor 2 11%
Student > Master 2 11%
Student > Ph. D. Student 2 11%
Other 4 21%
Unknown 3 16%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 8 42%
Neuroscience 3 16%
Medicine and Dentistry 3 16%
Business, Management and Accounting 1 5%
Unspecified 1 5%
Other 1 5%
Unknown 2 11%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 2. 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 14 August 2018.
All research outputs
#13,932,828
of 23,098,660 outputs
Outputs from Frontiers in Physiology
#4,912
of 13,846 outputs
Outputs of similar age
#175,968
of 330,334 outputs
Outputs of similar age from Frontiers in Physiology
#233
of 486 outputs
Altmetric has tracked 23,098,660 research outputs across all sources so far. This one is in the 38th percentile – i.e., 38% of other outputs scored the same or lower than it.
So far Altmetric has tracked 13,846 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.6. This one has gotten more attention than average, scoring higher than 64% 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 330,334 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 45th percentile – i.e., 45% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 486 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 51% of its contemporaries.