Evidence for mast cells contributing to neuromuscular pathology in an inherited model of ALS.

No abstract is available for this paper.

• Publication year

  2017

• Venue

  JCI Insight

• Publication date

  2017-10-19

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1172/jci.insight.95934PMID 29046475PMCID PMC5846907
• 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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  2014cited by this paper
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  2013cited by this paper
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  2012cited by this paper
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  2012cited by this paper
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  2012cited by this paper
• Modulating inflammatory monocytes with a unique microRNA gene signature ameliorates murine ALS.

  2012cited by this paper
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• Phenotypically aberrant astrocytes that promote motoneuron damage in a model of inherited amyotrophic lateral sclerosis

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• Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease.

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• Inflammation in amyotrophic lateral sclerosis spinal cord and brain is mediated by activated macrophages, mast cells and T cells

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• Skeletal muscle properties in a transgenic mouse model for amyotrophic lateral sclerosis: effects of creatine treatment.

  2003cited by this paper
• Nerve growth factor and eosinophils in inflamed juvenile conjunctival nevus.

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  2002influential reference
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  1997cited by this paper
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  1997cited by this paper
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  1996influential reference
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  1996cited by this paper
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  1996cited by this paper
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  1996cited by this paper
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  1995cited by this paper
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  1994cited by this paper
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  1994cited by this paper
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  1994cited by this paper
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  1993cited by this paper
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  1992cited by this paper
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  1990cited by this paper
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  1985cited by this paper
• Geometry of the capillary network in skeletal muscle.

  1976cited by this paper

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