1khb: Difference between revisions

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{{Seed}}
[[Image:1khb.png|left|200px]]
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{{STRUCTURE_1khb|  PDB=1khb  |  SCENE=  }}  
{{STRUCTURE_1khb|  PDB=1khb  |  SCENE=  }}  
===PEPCK complex with nonhydrolyzable GTP analog, native data===
===PEPCK complex with nonhydrolyzable GTP analog, native data===
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{{ABSTRACT_PUBMED_11851336}}
{{ABSTRACT_PUBMED_11851336}}


==Disease==
==Disease==
Known disease associated with this structure: Hypoglycemia due to PCK1 deficiency (1) OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=261680 261680]]
[[http://www.uniprot.org/uniprot/PCKGC_HUMAN PCKGC_HUMAN]] Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (C-PEPCKD) [MIM:[http://omim.org/entry/261680 261680]]. A metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait.  
 
==Function==
[[http://www.uniprot.org/uniprot/PCKGC_HUMAN PCKGC_HUMAN]] Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle.


==About this Structure==
==About this Structure==
1KHB is a 1 chain 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=1KHB OCA].  
[[1khb]] is a 1 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=1KHB OCA].  


==Reference==
==Reference==
<ref group="xtra">PMID:11851336</ref><references group="xtra"/>
<ref group="xtra">PMID:011851336</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Belunis, C.]]
[[Category: Belunis, C.]]
Line 40: Line 27:
[[Category: Wertheimer, S J.]]
[[Category: Wertheimer, S J.]]
[[Category: Gluconeogenesis]]
[[Category: Gluconeogenesis]]
[[Category: Lyase]]
[[Category: P-loop]]
[[Category: P-loop]]
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Feb 18 06:45:07 2009''
Retrieved from "https://proteopedia.org/1khb"