1azv

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[[Image:1azv.gif|left|200px]]
[[Image:1azv.gif|left|200px]]
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{{Structure
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<!--
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|PDB= 1azv |SIZE=350|CAPTION= <scene name='initialview01'>1azv</scene>, resolution 1.9&Aring;
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The line below this paragraph, containing "STRUCTURE_1azv", creates the "Structure Box" on the page.
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|SITE=
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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|LIGAND= <scene name='pdbligand=CU:COPPER+(II)+ION'>CU</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene>
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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|ACTIVITY= <span class='plainlinks'>[http://en.wikipedia.org/wiki/Superoxide_dismutase Superoxide dismutase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.15.1.1 1.15.1.1] </span>
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or leave the SCENE parameter empty for the default display.
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|GENE= SOD1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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-->
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|DOMAIN=
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{{STRUCTURE_1azv| PDB=1azv | SCENE= }}
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|RELATEDENTRY=
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1azv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1azv OCA], [http://www.ebi.ac.uk/pdbsum/1azv PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1azv RCSB]</span>
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}}
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'''FAMILIAL ALS MUTANT G37R CUZNSOD (HUMAN)'''
'''FAMILIAL ALS MUTANT G37R CUZNSOD (HUMAN)'''
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[[Category: Pellegrini, M.]]
[[Category: Pellegrini, M.]]
[[Category: Valentine, J S.]]
[[Category: Valentine, J S.]]
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[[Category: familial amyotrophic lateral sclerosis mutant]]
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[[Category: Familial amyotrophic lateral sclerosis mutant]]
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[[Category: oxidoreductase]]
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[[Category: Oxidoreductase]]
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[[Category: superoxide acceptor]]
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[[Category: Superoxide acceptor]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 10:53:41 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 18:50:47 2008''
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Revision as of 07:53, 2 May 2008

Image:1azv.gif

Template:STRUCTURE 1azv

FAMILIAL ALS MUTANT G37R CUZNSOD (HUMAN)


Contents

Overview

The X-ray crystal structure of a human copper/zinc superoxide dismutase mutant (G37R CuZnSOD) found in some patients with the inherited form of Lou Gehrig's disease (FALS) has been determined to 1.9 angstroms resolution. The two SOD subunits have distinct environments in the crystal and are different in structure at their copper binding sites. One subunit (subunit[intact]) shows a four-coordinate ligand geometry of the copper ion, whereas the other subunit (subunit[broken]) shows a three-coordinate geometry of the copper ion. Also, subunit(intact) displays higher atomic displacement parameters for backbone atoms ((B) = 30 +/- 10 angstroms2) than subunit(broken) ((B) = 24 +/- 11 angstroms2). This structure is the first CuZnSOD to show large differences between the two subunits. Factors that may contribute to these differences are discussed and a possible link of a looser structure to FALS is suggested.

Disease

Known disease associated with this structure: Amyotrophic lateral sclerosis, due to SOD1 deficiency OMIM:[147450]

About this Structure

1AZV is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis., Hart PJ, Liu H, Pellegrini M, Nersissian AM, Gralla EB, Valentine JS, Eisenberg D, Protein Sci. 1998 Mar;7(3):545-55. PMID:9541385 Page seeded by OCA on Fri May 2 10:53:41 2008

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OCA

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