Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:2.7.13.3 (histidine kinase)
 2,405 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The subunit dissociation of bovine liver glutamate dehydrogenase (L-glutamate: NAD(P)+ oxidoreductase (deaminating), EC 1.4.1.3) induced by guanidine hydrochloride ( GdnHCl ) in 0.2 M phosphate buffer (pH 7.3) was investigated by light-scattering molecular-weight measurements. With increasing GdnHCl concentration, two-step transition was observed in the molecular weight change. The dissociation behavior was well described by assuming the dissociation-association equilibria expressed as HK1 in equilibrium 2T K2 in equilibrium 6M where H, T, and M represent the hexameric, trimeric and monomeric forms of the enzyme, respectively. GdnHCl concentration dependence of the two equilibrium constants was interpreted in terms of the binding of GdnHCl on the protein. According to this treatment, the numbers of amino acid residues present at the trimer-trimer contact area within hexamer, N3, and at the monomer-monomer contact area within trimer, N1, were estimated to be as follows; N3 = 21 +/- 2 and N1 = 27 +/- 5. These values seem to be reasonable considering the physical model proposed for this enzyme.
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PMID:Light-scattering study on subunit association-dissociation equilibria of bovine liver glutamate dehydrogenase. 672 67

HP1043 of Helicobacter pylori is an orphan response regulator (RR) with a highly degenerate receiver sequence incapable of phosphorylation, which is essential for cell viability. In contrast, the orthologous RR protein of Helicobacter pullorum, an enterohepatic Helicobacter species mainly isolated from poultry, harbours a consensus receiver sequence and is associated with a cognate histidine kinase (HK). Here, we show that this two-component system of H. pullorum, denoted HPMG439/HPMG440, is involved in the control of nitrogen metabolism by regulating the expression of glutamate dehydrogenase, an AmtB ammonium transporter and a PII protein. However, the role of the RR HPMG439 is not restricted to nitrogen regulation since, in contrast with the HK HPMG440, HPMG439 is essential for growth of H. pullorum under nutrient-rich conditions.
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PMID:Novel function assignment to a member of the essential HP1043 response regulator family of epsilon-proteobacteria. 2347 51

Congenital hyperinsulinism (CHI) is a clinically and genetically heterogeneous disorder, characterized by dysregulated insulin secretion. Pathogenic variants in at least twelve different genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF4A, HNF1A, UCP2, TRMT10A HK1, and PGM1) are known to cause CHI. Pathogenic variants in the GLUD1 gene, which encodes the enzyme glutamate dehydrogenase (GDH), account for 5% of the cases of congenital hyperinsulinemic hypoglycemia. Pathogenic variants in GLUD1 typically present in late infancy, are diet and/or diazoxide-responsive and cause protein-induced hyperinsulinemic hypoglycemia as insulin secretion is triggered by allosteric activation of GDH by leucine. The authors are presenting three unrelated Indian children, who manifested with fasting as well as dietary protein induced hypoglycemia in late infancy, and were diagnosed to have hyperinsulinemic hyperammonemic hypoglycemia due to pathogenic variants in GLUD1. Although the hypoglycemia responded to diazoxide, delayed diagnosis and irregular treatment had resulted in neurological problems in two of the three children. Early identification, appropriate dietary modifications and regular treatment with diazoxide can prevent adverse neurological outcome.
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PMID:Congenital Hyperinsulinemic Hypoglycemia and Hyperammonemia due to Pathogenic Variants in GLUD1. 3111 23