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1khe
From Proteopedia
Contents |
PEPCK complex with nonhydrolyzable GTP analog, MAD 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
1khe 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
