Crystal structure of human wildtype and S581L‐mutant glycyl‐tRNA synthetase, an enzyme underlying distal spinal muscular atrophy

No abstract is available for this paper.

• Publication year

  2007

• Venue

  FEBS Letters

• Publication date

  2007-06-26

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.febslet.2007.05.046PMID 17544401
• 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.

• The G526R glycyl-tRNA synthetase gene mutation in distal hereditary motor neuropathy type V

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• Editing-defective tRNA synthetase causes protein misfolding and neurodegeneration

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• Severe childhood SMA and axonal CMT due to anticodon binding domain mutations in the GARS gene

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  1995influential reference
• Mapping of a distal form of spinal muscular atrophy with upper limb predominance to chromosome 7p.

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• Associations between Neurological Diseases and Mutations in the Human Glycyl-tRNA Synthetase

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  2015cites this paper
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