2q5i: Difference between revisions

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{{STRUCTURE_2q5i|  PDB=2q5i  |  SCENE=  }}  
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'''Crystal structure of apo S581L Glycyl-tRNA synthetase mutant'''
===Crystal structure of apo S581L Glycyl-tRNA synthetase mutant===




==Overview==
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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 could indirectly affect glycine binding via the tRNA or alternatively inhibit conformational changes. Reduced enzyme activity may underlie neuronal degeneration, although a dominant-negative effect is more likely in this autosomal dominant disorder.
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==Disease==
==Disease==
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[[Category: Oxford protein production facility]]
[[Category: Oxford protein production facility]]
[[Category: Structural genomic]]
[[Category: Structural genomic]]
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