3bg3
From Proteopedia
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{{STRUCTURE_3bg3| PDB=3bg3 | SCENE= }} | {{STRUCTURE_3bg3| PDB=3bg3 | SCENE= }} | ||
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===Crystal Structure of Human Pyruvate Carboxylase (missing the biotin carboxylase domain at the N-terminus)=== | ===Crystal Structure of Human Pyruvate Carboxylase (missing the biotin carboxylase domain at the N-terminus)=== | ||
| + | {{ABSTRACT_PUBMED_18297087}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/PYC_HUMAN PYC_HUMAN]] Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:[http://omim.org/entry/266150 266150]]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia. | ||
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| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/PYC_HUMAN PYC_HUMAN]] Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate. | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:018297087</ref><references group="xtra"/> | + | <ref group="xtra">PMID:018297087</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Pyruvate carboxylase]] | [[Category: Pyruvate carboxylase]] | ||
Revision as of 07:22, 25 March 2013
Contents |
Crystal Structure of Human Pyruvate Carboxylase (missing the biotin carboxylase domain at the N-terminus)
Template:ABSTRACT PUBMED 18297087
Disease
[PYC_HUMAN] Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia.
Function
[PYC_HUMAN] Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.
About this Structure
3bg3 is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Xiang S, Tong L. Crystal structures of human and Staphylococcus aureus pyruvate carboxylase and molecular insights into the carboxyltransfer reaction. Nat Struct Mol Biol. 2008 Mar;15(3):295-302. Epub 2008 Feb 24. PMID:18297087 doi:10.1038/nsmb.1393
