Phosphoglycerate kinase activation

Bernstein BE, Michels PAM, Hoi WGJ. Synergistic effects of substrate-induced conformational changes in phosphoglycerate kinase activation. Nature 1997 385 275-278. 

Bernstein, B.E. Hoi, W.G. Crystal structures of substrates and products bound to the phosphoglycerate kinase active site reveal the catalytic mechanism. Biochemistry, 37, 4429-4436 (1998)... 

Hereditary deficiency of phosphoglycerate kinase (PGK) is associated with hereditary hemolytic anemia and often with central nervous system dysfunction and/or myopathy. The first case, reported by Kraus et al. (K24), is a heterozygous female, and the results are not so clear. The second family, reported by Valentine et al. (V3), is a large Chinese family, whose pedigree study indicates that PGK deficiency is compatible with X-linked inheritance. To date, 22 families have been reported (04, T25, Y3). Nine of these have manifested both symptoms five have shown only hemolysis seven have shown the central nervous system dysfunction and/or myopathy but without hemolysis and one case, PGK Munchen, is without clinical symptoms (F5). PGK II is an electrophoretic variant found in New Guinea populations (Y2). Red blood cell enzyme activity, specific activity, and the kinetic properties of this polymorphic variant are normal. 

ATP Triphosphate Chain Conformation. Much of the work in the area of ATP triphosphate chain conformation has been performed by Cleland and co-workers (14--16). Their studies on metal(III)ATP interactions with kinases have led to the classification of kinases according to the stereochemistry of the polyphosphate chain as it binds to the active site. For the kinases they studied (hexokinase, glycerokinase, creatine kinase, phosphofructokinase, 3-phosphoglycerate kinase, acetate kinase, arginine kinase, adenylate kinase and pyruvate kinase) it was found that B, y-bidentate chromi M(III)-ATP (CrATP) and not a,6,y-tridentate CrATP is a... 

Joao HC, Williams RJP, Littlechild JA, Nagasuma R, Watson HC. An investigation of large inhibitors binding to phosphoglycerate kinase and their effect on anion activation. Eur J Biochem 1992 205 1077-1088. 

Larsson-Raznikiewicz, M. Schierbeck, B. Activation and inhibition of the phosphoglycerate kinase reaction by ATP. Biochim. Biophys. Acta, 481, 283-287 (1977)... 

Szilagyi, A.N. Vas, M. Anion activation of 3-phosphoglycerate kinase requires domain closure. Biochemistry, 37, 8551-8563 (1998)... 

Bentahir, M. Feller, G. Aittaleb, M. Lamotte-Brasseur, J. Himri, T. Chessa, J.P. Gerday, C. Structural, kinetic, and calorimetric characterization of the cold-active phosphoglycerate kinase from the antarctic Pseudomonas sp. TACII18. J. Biol. Chem., 275, 11147-11153 (2000)... 

Phosphoglycerate kinase is encoded by the mammalian X chromosome. Several mutant forms are associated with hemolytic anemia and mental disorders.885 886 Mutation of Glu 190, which is in the hinge region far from the active site, to Gin or Asn markedly reduces enzymatic activity.887... 

Fig. 5. The structure of the enzyme active site of phosphoglycerate kinase deduced without knowledge of the X-ray structure see Refs. 26 und 27 PGA is phospho-glycerate - the second substrate, numbers refer to individual resonances studied. O N O water... 

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