1m7r

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[[Image:1m7r.jpg|left|200px]]
[[Image:1m7r.jpg|left|200px]]
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{{Structure
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|PDB= 1m7r |SIZE=350|CAPTION= <scene name='initialview01'>1m7r</scene>, resolution 2.60&Aring;
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The line below this paragraph, containing "STRUCTURE_1m7r", creates the "Structure Box" on the page.
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|SITE=
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|LIGAND= <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</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/Phosphatidylinositol-3-phosphatase Phosphatidylinositol-3-phosphatase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.1.3.64 3.1.3.64] </span>
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|GENE= MTMR2 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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|DOMAIN=
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{{STRUCTURE_1m7r| PDB=1m7r | SCENE= }}
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|RELATEDENTRY=[[1lw3|1LW3]]
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1m7r FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1m7r OCA], [http://www.ebi.ac.uk/pdbsum/1m7r PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1m7r RCSB]</span>
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'''Crystal Structure of Myotubularin-related Protein-2 (MTMR2) Complexed with Phosphate'''
'''Crystal Structure of Myotubularin-related Protein-2 (MTMR2) Complexed with Phosphate'''
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[[Category: Taylor, G S.]]
[[Category: Taylor, G S.]]
[[Category: Veine, D M.]]
[[Category: Veine, D M.]]
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[[Category: protein-phosphate complex]]
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[[Category: Protein-phosphate complex]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 00:44:20 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 22:12:33 2008''
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Revision as of 21:44, 2 May 2008

Template:STRUCTURE 1m7r

Crystal Structure of Myotubularin-related Protein-2 (MTMR2) Complexed with Phosphate


Contents

Overview

Myotubularin-related proteins are a large subfamily of protein tyrosine phosphatases (PTPs) that dephosphorylate D3-phosphorylated inositol lipids. Mutations in members of the myotubularin family cause the human neuromuscular disorders myotubular myopathy and type 4B Charcot-Marie-Tooth syndrome. The crystal structure of a representative member of this family, MTMR2, reveals a phosphatase domain that is structurally unique among PTPs. A series of mutants are described that exhibit altered enzymatic activity and provide insight into the specificity of myotubularin phosphatases toward phosphoinositide substrates. The structure also reveals that the GRAM domain, found in myotubularin family phosphatases and predicted to occur in approximately 180 proteins, is part of a larger motif with a pleckstrin homology (PH) domain fold. Finally, the MTMR2 structure will serve as a model for other members of the myotubularin family and provide a framework for understanding the mechanism whereby mutations in these proteins lead to disease.

Disease

Known disease associated with this structure: Charcot-Marie-Tooth disease, type 4B1 OMIM:[603557]

About this Structure

1M7R is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Crystal structure of a phosphoinositide phosphatase, MTMR2: insights into myotubular myopathy and Charcot-Marie-Tooth syndrome., Begley MJ, Taylor GS, Kim SA, Veine DM, Dixon JE, Stuckey JA, Mol Cell. 2003 Dec;12(6):1391-402. PMID:14690594 Page seeded by OCA on Sat May 3 00:44:20 2008

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