3lp4

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[[Image:3lp4.png|left|200px]]
 
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{{STRUCTURE_3lp4| PDB=3lp4 | SCENE= }}
{{STRUCTURE_3lp4| PDB=3lp4 | SCENE= }}
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===Crystal structure of human arginase I in complex with L-LYSINE, 1.90A Resolution.===
===Crystal structure of human arginase I in complex with L-LYSINE, 1.90A Resolution.===
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{{ABSTRACT_PUBMED_20153713}}
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==Disease==
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[[http://www.uniprot.org/uniprot/ARGI1_HUMAN ARGI1_HUMAN]] Defects in ARG1 are the cause of argininemia (ARGIN) [MIM:[http://omim.org/entry/207800 207800]]; also known as hyperargininemia. Argininemia is a rare autosomal recessive disorder of the urea cycle. Arginine is elevated in the blood and cerebrospinal fluid, and periodic hyperammonemia occurs. Clinical manifestations include developmental delay, seizures, mental retardation, hypotonia, ataxia, progressive spastic quadriplegia.<ref>PMID:1463019</ref><ref>PMID:7649538</ref>
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{{ABSTRACT_PUBMED_20153713}}
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==About this Structure==
==About this Structure==
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3LP4 is a 2 chains structure with sequences 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=3LP4 OCA].
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[[3lp4]] is a 2 chain structure with 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=3LP4 OCA].
==Reference==
==Reference==
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<ref group="xtra">PMID:20153713</ref><references group="xtra"/>
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<ref group="xtra">PMID:020153713</ref><references group="xtra"/><references/>
[[Category: Arginase]]
[[Category: Arginase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Costanzo, L Di.]]
[[Category: Costanzo, L Di.]]
[[Category: Arginine metabolism]]
[[Category: Arginine metabolism]]
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[[Category: Cytoplasm]]
 
[[Category: Hydrolase]]
[[Category: Hydrolase]]
[[Category: Lysine inhibition]]
[[Category: Lysine inhibition]]
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[[Category: Metal-binding]]
[[Category: Metal-binding]]
[[Category: Urea cycle]]
[[Category: Urea cycle]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Jun 10 10:53:15 2010''
 

Revision as of 03:37, 25 March 2013

Template:STRUCTURE 3lp4

Contents

Crystal structure of human arginase I in complex with L-LYSINE, 1.90A Resolution.

Template:ABSTRACT PUBMED 20153713

Disease

[ARGI1_HUMAN] Defects in ARG1 are the cause of argininemia (ARGIN) [MIM:207800]; also known as hyperargininemia. Argininemia is a rare autosomal recessive disorder of the urea cycle. Arginine is elevated in the blood and cerebrospinal fluid, and periodic hyperammonemia occurs. Clinical manifestations include developmental delay, seizures, mental retardation, hypotonia, ataxia, progressive spastic quadriplegia.[1][2]

About this Structure

3lp4 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Di Costanzo L, Ilies M, Thorn KJ, Christianson DW. Inhibition of human arginase I by substrate and product analogues. Arch Biochem Biophys. 2010 Apr 15;496(2):101-8. Epub 2010 Feb 12. PMID:20153713 doi:10.1016/j.abb.2010.02.004
  1. Uchino T, Haraguchi Y, Aparicio JM, Mizutani N, Higashikawa M, Naitoh H, Mori M, Matsuda I. Three novel mutations in the liver-type arginase gene in three unrelated Japanese patients with argininemia. Am J Hum Genet. 1992 Dec;51(6):1406-12. PMID:1463019
  2. Uchino T, Snyderman SE, Lambert M, Qureshi IA, Shapira SK, Sansaricq C, Smit LM, Jakobs C, Matsuda I. Molecular basis of phenotypic variation in patients with argininemia. Hum Genet. 1995 Sep;96(3):255-60. PMID:7649538

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