2q5h: Difference between revisions
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[[Image:2q5h.jpg|left|200px]] | [[Image:2q5h.jpg|left|200px]] | ||
'''Crystal structure of apo-wildtype Glycyl-tRNA synthetase''' | {{Structure | ||
|PDB= 2q5h |SIZE=350|CAPTION= <scene name='initialview01'>2q5h</scene>, resolution 3.0Å | |||
|SITE= | |||
|LIGAND= | |||
|ACTIVITY= [http://en.wikipedia.org/wiki/Glycine--tRNA_ligase Glycine--tRNA ligase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=6.1.1.14 6.1.1.14] | |||
|GENE= GARS ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]) | |||
}} | |||
'''Crystal structure of apo-wildtype Glycyl-tRNA synthetase''' | |||
==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
2Q5H is a [ | 2Q5H is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2Q5H OCA]. | ||
==Reference== | ==Reference== | ||
Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy., Cader MZ, Ren J, James PA, Bird LE, Talbot K, Stammers DK, FEBS Lett. 2007 Jun 26;581(16):2959-64. Epub 2007 May 29. PMID:[http:// | Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy., Cader MZ, Ren J, James PA, Bird LE, Talbot K, Stammers DK, FEBS Lett. 2007 Jun 26;581(16):2959-64. Epub 2007 May 29. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/17544401 17544401] | ||
[[Category: Glycine--tRNA ligase]] | [[Category: Glycine--tRNA ligase]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
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[[Category: oppf]] | [[Category: oppf]] | ||
[[Category: oxford protein production facility]] | [[Category: oxford protein production facility]] | ||
[[Category: structural | [[Category: structural genomic]] | ||
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 18:21:56 2008'' | ||
Revision as of 19:21, 20 March 2008
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| , resolution 3.0Å | |||||||
|---|---|---|---|---|---|---|---|
| Gene: | GARS (Homo sapiens) | ||||||
| Activity: | Glycine--tRNA ligase, with EC number 6.1.1.14 | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
Crystal structure of apo-wildtype Glycyl-tRNA synthetase
OverviewOverview
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.
DiseaseDisease
Known diseases associated with this structure: Charcot-Marie-Tooth disease, type 2D OMIM:[600287], Neuropathy, distal hereditary motor, type V OMIM:[600287]
About this StructureAbout this Structure
2Q5H is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy., Cader MZ, Ren J, James PA, Bird LE, Talbot K, Stammers DK, FEBS Lett. 2007 Jun 26;581(16):2959-64. Epub 2007 May 29. PMID:17544401
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