1khb
From Proteopedia
| Line 1: | Line 1: | ||
| - | {{Seed}} | ||
| - | [[Image:1khb.png|left|200px]] | ||
| - | |||
| - | <!-- | ||
| - | The line below this paragraph, containing "STRUCTURE_1khb", creates the "Structure Box" on the page. | ||
| - | You may change the PDB parameter (which sets the PDB file loaded into the applet) | ||
| - | or the SCENE parameter (which sets the initial scene displayed when the page is loaded), | ||
| - | or leave the SCENE parameter empty for the default display. | ||
| - | --> | ||
{{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=== | ||
| - | |||
| - | |||
| - | <!-- | ||
| - | The line below this paragraph, {{ABSTRACT_PUBMED_11851336}}, adds the Publication Abstract to the page | ||
| - | (as it appears on PubMed at http://www.pubmed.gov), where 11851336 is the PubMed ID number. | ||
| - | --> | ||
{{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]] | ||
| - | |||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Feb 18 06:45:07 2009'' | ||
Revision as of 02:53, 25 March 2013
Contents |
PEPCK complex with nonhydrolyzable GTP analog, native data
Template:ABSTRACT PUBMED 11851336
Disease
[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.
Function
[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
1khb is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- 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
Categories: Homo sapiens | Belunis, C. | Crowther, R. | Dunten, P. | Hollfelder, K. | Kammlott, U. | Levin, W. | Michel, H. | Ramsey, G B. | Swain, A. | Weber, D. | Wertheimer, S J. | Gluconeogenesis | Lyase | P-loop
