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

Abstract:

Dominant mutations in the ubiquitous enzyme glycyl-tRNA synthetase (GlyRS), including S581L, lead to motor nerve degeneration. We have determined crystal structures of wildtype and S581L-mutant human GlyRS. The S581L mutation is approximately 50A from the active site, and yet gives reduced aminoacylation activity. The overall structures of wildtype and S581L-GlyRS, including the active site, are very similar. However, residues 567-575 of the anticodon-binding domain shift position and in turn...

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Publication status:
Published

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Institution:
University of Oxford
Department:
Oxford, MSD, Clinical Neuroscience, Molecular Medicine
Role:
Author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, Clinical Neuroscience
Role:
Author
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Journal:
FEBS letters
Volume:
581
Issue:
16
Pages:
2959-2964
Publication date:
2007-06-05
DOI:
EISSN:
1873-3468
ISSN:
0014-5793
URN:
uuid:ea618814-ccae-43bc-80d2-9bd8d5d35004
Source identifiers:
72646
Local pid:
pubs:72646

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