The glucokinase regulatory protein (GKRP) plays an essential role in glucose homeostasis by acting as a competitive inhibitor of glucokinase (GCK) and triggering its localization to the hepatocyte nucleus upon glucose deprivation. Metabolites such as fructose 6-phosphate and sorbitol 6-phosphate promote assembly of the GCK-GKRP complex, whereas fructose 1-phosphate and functionalized piperazines with potent in vivo antidiabetic activity disrupt the complex. Here, we establish the molecular basis by which these natural and synthetic ligands modulate the GCK-GKRP interaction. We demonstrate that a small-molecule disruptor of the protein-protein interaction utilizes a two-step conformational selection mechanism to associate with a rare GKRP conformation constituting 3% of the total population. Conformational heterogeneity of GKRP is localized to the N-terminus and deleting this region eliminates the ability of sorbitol 6-phosphate to promote the GCK-GKRP interaction. Stabilizing ligands favor an extended N-terminus, which sterically positions two arginine residues for optimal Coulombic interaction with a pair of carboxylate side chains from GCK. Conversely, disruptors promote a more compact N-terminus in which an interfacial arginine residue is stabilized in an unproductive orientation through a cation-π interaction with tyrosine 75. Eliminating the ability to sample this binding impaired conformation enhances the intrinsic inhibitory activity of GKRP. Elucidating the molecular basis of ligand-mediated control over the GCK-GKRP interaction is expected to impact the development and future refinement of therapeutic agents for diabetes and cardiovascular disease, which result from improper GKRP regulation of GCK.
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http://dx.doi.org/10.1021/acs.biochem.7b00377 | DOI Listing |
Biochim Biophys Acta Mol Basis Dis
July 2018
Departamento de Bioquímica y Biología Molecular, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain; Centro de Investigación Biomédica en Red de Diabetes y Enfermedades Metabólicas (CIBERDEM), www.ciberdem.net, Instituto de Salud Carlos III, Madrid, Spain; Instituto de Investigación Sanitaria del Hospital Clínico San Carlos (IdISSC), Madrid, Spain. Electronic address:
Glucokinase (GCK) plays a key role in glucose homeostasis. Heterozygous inactivating mutations in the GCK gene cause the familial, mild fasting hyperglycaemia named MODY2. Besides its particular kinetic characteristics, glucokinase is regulated by subcellular compartmentation in hepatocytes.
View Article and Find Full Text PDFBiochemistry
June 2017
Department of Chemistry and Biochemistry, Florida State University, Tallahassee, Florida 32303, United States.
The glucokinase regulatory protein (GKRP) plays an essential role in glucose homeostasis by acting as a competitive inhibitor of glucokinase (GCK) and triggering its localization to the hepatocyte nucleus upon glucose deprivation. Metabolites such as fructose 6-phosphate and sorbitol 6-phosphate promote assembly of the GCK-GKRP complex, whereas fructose 1-phosphate and functionalized piperazines with potent in vivo antidiabetic activity disrupt the complex. Here, we establish the molecular basis by which these natural and synthetic ligands modulate the GCK-GKRP interaction.
View Article and Find Full Text PDFExpert Opin Ther Targets
September 2016
a Division of Diabetes, Endocrinology and Metabolism , Tokyo Medical University, 6-7-1Nishi-shinjuku, Shinjuku-ku , Tokyo 160-0023 , Japan.
Introduction: Type 2 diabetes (T2D) is a complex disease caused by an interaction between multiple genetic and environmental factors. T2D-associated loci identified by genome-wide association studies (GWAS) harbor the genes targeted by many clinically available drugs, supporting the idea that GWAS have the potential to discover novel genes for drug development.
Areas Covered: This paper outlines, among the genes at those T2D-associated loci, the functional analysis of FTO, TCF7L2, SLC30A8, and MTNR1B, illustrating caveats we should be cautious about.
Curr Opin Lipidol
April 2015
aOxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK bCenter for the Science of Therapeutics, Broad Institute cHoward Hughes Medical Institute, Broad Institute, Cambridge, Massachusetts, USA dNIHR Oxford Biomedical Research Centre, ORH Trust, OCDEM, Churchill Hospital, Oxford, UK.
Purpose Of Review: Glucokinase regulator (GCKR) encodes glucokinase regulatory protein (GKRP), a hepatocyte-specific inhibitor of the glucose-metabolizing enzyme glucokinase (GCK). Genome-wide association studies have identified a common coding variant within GCKR associated with multiple metabolic traits. This review focuses on recent insights into the critical role of GKRP in hepatic glucose metabolism that have stemmed from the study of human genetics.
View Article and Find Full Text PDFPLoS One
October 2014
National Center for Advancing Translational Sciences, National Institutes of Health, Rockville, Maryland, United States of America.
Recent genetic and clinical evidence has implicated glucokinase regulatory protein (GKRP) in the pathogenesis of type 2 diabetes and related traits. The primary role of GKRP is to bind and inhibit hepatic glucokinase (GCK), a critically important protein in human health and disease that exerts a significant degree of control over glucose metabolism. As activation of GCK has been associated with improved glucose tolerance, perturbation of the GCK-GKRP interaction represents a potential therapeutic target for pharmacological modulation.
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