1khb: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
No edit summary |
||
| Line 1: | Line 1: | ||
{{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=== | ||
{{ABSTRACT_PUBMED_11851336}} | {{ABSTRACT_PUBMED_11851336}} | ||
==Disease== | ==Disease== | ||
[[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 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: | <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]] | ||
Revision as of 05:53, 25 March 2013
PEPCK complex with nonhydrolyzable GTP analog, native dataPEPCK complex with nonhydrolyzable GTP analog, native data
Template:ABSTRACT PUBMED 11851336
DiseaseDisease
[PCKGC_HUMAN] Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (C-PEPCKD) [MIM: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.
FunctionFunction
[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 StructureAbout this Structure
1khb is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
- ↑ Dunten P, Belunis C, Crowther R, Hollfelder K, Kammlott U, Levin W, Michel H, Ramsey GB, Swain A, Weber D, Wertheimer SJ. Crystal structure of human cytosolic phosphoenolpyruvate carboxykinase reveals a new GTP-binding site. J Mol Biol. 2002 Feb 15;316(2):257-64. PMID:11851336 doi:http://dx.doi.org/10.1006/jmbi.2001.5364