Modeling Huntington׳s disease with patient-derived neurons.

No abstract is available for this paper.

• Publication year

  2017

• Venue

  Brain Research

• Publication date

  2017-02-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.brainres.2015.10.001PMID 26459990
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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  2015influential reference
• Mutant huntingtin downregulates myelin regulatory factor-mediated myelin gene expression and affects mature oligodendrocytes.

  2015cited by this paper
• The Neuropathology of Huntington's Disease.

  2015cited by this paper
• Increased Risk of Genetic and Epigenetic Instability in Human Embryonic Stem Cells Associated with Specific Culture Conditions

  2015cited by this paper
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  2015cited by this paper
• Review: The fate of cell grafts for the treatment of Huntington's disease: the post‐mortem evidence

  2014cited by this paper
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  2014cited by this paper
• Proteomics of Huntington's disease-affected human embryonic stem cells reveals an evolving pathology involving mitochondrial dysfunction and metabolic disturbances.

  2014influential reference
• All Roads Lead to Induced Pluripotent Stem Cells: The Technologies of iPSC Generation

  2014cited by this paper
• Microfluidic organs-on-chips

  2014cited by this paper
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  2014influential reference
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  2014cited by this paper
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  2014cited by this paper
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  2013cited by this paper
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  2013cited by this paper
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  2013influential reference
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  2013cited by this paper
• EZ spheres: a stable and expandable culture system for the generation of pre-rosette multipotent stem cells from human ESCs and iPSCs.

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  2013influential reference
• Cerebral organoids model human brain development and microcephaly

  2013cited by this paper
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  2013influential reference
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  2012influential reference
• Directed differentiation of human pluripotent stem cells to cerebral cortex neurons and neural networks

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• Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells.

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• Genetic correction of Huntington's disease phenotypes in induced pluripotent stem cells.

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  2012influential reference
• The incidence and prevalence of Huntington's disease: A systematic review and meta‐analysis

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  2011cited by this paper
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  2011cited by this paper
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  2011cited by this paper
• Induced pluripotent stem cells (iPSCs) and neurological disease modeling: progress and promises.

  2011cited by this paper
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  2011cited by this paper
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  2010influential reference
• Graft and host interactions following transplantation of neural stem cells to organotypic striatal cultures.

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  2010cited by this paper
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  2010cited by this paper
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  2009influential reference
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  2009cited by this paper
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  2009cited by this paper
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  2009influential reference
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  2009cited by this paper
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  2008influential reference
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  2008cited by this paper

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  2021cites this paper
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  2019cites this paper
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  2019cites this paper
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  2019cites this paper
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  2019cites this paper
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  2018cites this paper
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  2018cites this paper
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  2018cites this paper
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  2018cites this paper
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  2017cites this paper
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