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  • 21
    ISSN: 1432-1076
    Keywords: Key words Glucosephosphate isomerase ; haemolytic anaemia ; enzyme mutants
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Biochemical and molecular genetic studies were performed on the enzyme variants of two patients compound heterozygous for glucose phosphate isomerase (GPI) deficiency, both suffering from severe haemolytic anaemia. The enzymes of case 1 (GPI `Zwickau') and case 2 (GPI `Nordhorn' [25]), revealed reduced GPI activity and remarkable thermolability. Glucose-6-phosphate (Gluc-6-P) concentration was elevated 2.3 times in case 1 and 3.8 times in case 2. Sequencing the patients' GPI genes showed four different point mutations, two of them involving highly conserved amino acids. The c1039 C→T substitution, found in the gene of GPI `Zwickau', has been described recently [30] and causes an Arg 347→Cys substitution close to the putative catalytic site. The second mutation in this case is a novel c1538 G→A substitution causing a Trp→stop mutation at position 513 apparently resulting in premature RNA degradation thus resulting either in a complete lack of protein or a protein which does not show GPI activity. In the gene of GPI `Nordhorn' a c1028 A→G mutation was discovered, also previously described [1, 9] causing a Gln 343→Trp substitution. The second mutation was a novel splice site mutation at the border of intron 15 to exon 16: IVS15-(-2) A→C which leads to an aberrant splicing of exon 16, thus resulting either in a truncated and most likely inactive enzyme or in no protein at all. Conclusion Biochemical and molecular genetic studies performed with the enzyme variants GPI `Zwickau' and GPI `Nordhorn' showed that in both cases the simultaneous occurrence of a single amino acid sub‐stitution affecting the active site, together with a nonsense mutation leading to the loss of major parts of the enzyme probably explains the severe clinical course of the disease.
    Type of Medium: Electronic Resource
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  • 22
    Electronic Resource
    Electronic Resource
    Springer
    European biophysics journal 16 (1988), S. 219-229 
    ISSN: 1432-1017
    Keywords: Relaxation kinetics ; ultrasound absorption ; temperature jump ; proton transfer ; vitamin B 6
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Physics
    Notes: Abstract UV-visible and 13C NMR measurements described in the literature and our 31P NMR measurements support the following mechanism of proton transfer reactions in aqueous solutions of pyridoxamine phosphate: Only the tautomeric equilibrium between neutral form, A N, and zwitterion, A Z, which is analogous to the tautomeric equilibrium of 3-hydroxypyridine in aqueous solution, is important, and that equilibrium does not change upon the dissociation of the second phosphate proton. With these simplifying assumption, we have simulated the relaxation spectrum of the proton transfer reactions of pyridoxamine phosphate in water using parameters from analogous reactions and compared it with our ultrasound and temperature jump measurements. We have found that the relaxation process measured by the temperature jump experiment is mainly caused by the overall reaction A N=A Z (or A N - =A Z - ) and the ultrasound absorption at the isoelectric point between pK2 and pK3 is mainly caused by the overall reaction $$A^{\text{ + }} + {\text{x}} A_N^ - + \left( {1 - {\text{x}}} \right)A_Z^ - = {\text{y}}A_N + \left( {2 - {\text{y}}} \right)A_Z , 0 \leqq {\text{x}} \leqq {\text{1,}} {\text{0}} \leqq {\text{y}} \leqq 2$$ .
    Type of Medium: Electronic Resource
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  • 23
    ISSN: 1432-1017
    Keywords: Key words Carbonmonoxy-Myoglobin ; Recombination kinetics ; Mössbauer spectroscopy ; Scaling law ; Activated tunneling
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Physics
    Notes: Abstract We have studied the recombination kinetics of carboxymyoglobin (after photodissociation of the CO ligand) by Mössbauer spectroscopy for temperatures in the range 4.2 – 60 K. The observed kinetics display non-exponential behaviour which was monitored over periods of a few days. It is shown that the time dependence of the kinetics can be reduced to a single universal function of the temperature-dependent variable (t/τ 1/2(T)) β(T) . The half-decay time τ 1/2(T) and the scaling parameter β(T) are analysed for the presence of tunneling effects. The non-Arrhenius temperature dependence of the half-decay time below 60 K is interpreted as activated tunneling in models with an Eckart barrier or a fluctuating barrier.
    Type of Medium: Electronic Resource
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  • 24
    Electronic Resource
    Electronic Resource
    Springer
    Il nuovo cimento della Società Italiana di Fisica 18 (1996), S. 353-357 
    ISSN: 0392-6737
    Keywords: Mössbauer spectra ; Spectra ; photodissociation and photoionization of biomolecules ; bioluminescence ; Measurement of rate constants ; reaction cross-sections and activation energy ; Conference proceedings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Summary Partial photodissociation of two carbonmonoxy-porphyrin complexes, a bridged and a fenced one, has been achieved. The results from monitoring the recombination point to an important influence of the solvent matrix on the recombination rates.
    Type of Medium: Electronic Resource
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  • 25
    ISSN: 0392-6737
    Keywords: Phonons and vibrations in crystal lattices ; Solid-liquid transitions ; Thermodynamic properties and entropy ; Conference proceedings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Summary Dynamic properties of ultrafine clusters of γ-Fe2O3 (ferric oxide, FO) were studied by Mössbauer spectroscopy and by thermodynamic analysis. The data obtained for FO clusters allowed the conclusion that dynamic properties of clusters as well as the decrease of melting point and the appearance of a gap between freezing and melting points depend on intracluster atomic mobility. Intracluster atomic mobility in FO clusters was shown to increase by the action of surfactants which decrease intercluster interactions. The increase in intracluster atomic mobility was suggested to proceed via formation of a solid-liquid state.
    Type of Medium: Electronic Resource
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  • 26
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 89 (1988), S. 732-740 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: The hyperfine coupling parameters of Fe(II) in the FeS4 unit of the complex anion [Fe(SC6H5)4]2− have been determined from an analysis of Mössbauer spectra of a monocrystal in the temperature range of 1.5 to 20 K and in magnetic fields of 0 to 6.7 T. Dynamic spin fluctuation effects have been included in the analysis by a stochastic model with spin–lattice relaxation. Anisotropy in the relaxation matrix element was of moderate significance and apparent only at low fields. The use of a monocrystal allows more precise determination of hyperfine parameters, because it avoids the averaging procedures which are inherent in studies on polycrystalline material. The set of parameters is essentially identical with that reported for reduced rubredoxin and indicates that the electronic ground state of [Fe(SC6H5)4]2−, predominantly of [3z2−r2] character, simulates very well the ground state of the protein FeS core.
    Type of Medium: Electronic Resource
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  • 27
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 82 (1985), S. 3584-3593 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: The electronic structures of mononuclear Fe–S complexes with a FeIIS4 core and of binuclear Fe–Mo–S complexes containing the FeS2Mo core have been calculated by a semiempirical molecular orbital method (iterative extended Hückel theory), followed by a spin-orbit coupling calculation on the five highest occupied iron-like molecular orbitals. Fine structure and hyperfine structure tensors and parameters (g, D, E, A, and electric field gradient) have been calculated and compared with data from spin-Hamiltonian analysis of Mössbauer measurements. For the mononuclear complex anions [Fe(SPh)4]2− and [Fe(dts)2]2− it was found that Vzˆzˆ is negative, D positive, and that the magnetic anisotropy places the preferred direction of the hyperfine magnetic field perpendicular to the Vzˆzˆ direction in agreement with spin-Hamiltonian results. The similarity of parameters of [Fe(SPh)4]2− and reduced rubredoxin (Rdred) confirms the suggestion that this anion has a ground electronic state practically identical to Rdred. The complex anion [Fe(dts)2]2− shows smaller anisotropy, and due to the fact that the orbital ground state is energetically not well separated from higher states in this case a strong temperature dependence of the quadrupole splitting is observed. For the binuclear complex anions [(SPh)2FeS2MoS2]2−, [S5FeS2MoS2]2−, and [Cl2FeS2MoS2]2− it was found that d is negative and Vzˆzˆ is positive. A specific feature of these binuclear Fe–Mo–S complexes is that Vzˆzˆ is directed perpendicular to the Fe–Mo line. (This theoretical result is confirmed by single crystal Mössbauer studies on [Cl2FeS2MoS2]2−; see the following paper in this journal.) The preferred direction of the magnetic hyperfine field is close to the Vzˆzˆ axis. The correlation of calculated values of ρ(0) and isomer shifts for mononuclear and binuclear compounds confirms the role of MoS2−4 as a charge withdrawing ligand.
    Type of Medium: Electronic Resource
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  • 28
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 82 (1985), S. 3594-3598 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: A single crystal of [(C6H5)4P][(C6H5CH2)–(CH3)3N][Cl2FeS2MoS2] has been investigated by Mössbauer spectroscopy with various orientations of the crystal with respect to the γ beam. From this investigation we derive that the main component of the electric field gradient tensor Vzˆzˆ is positive and oriented perpendicular to the Fe–Mo direction. This result was confirmed by additional single crystal measurements applying an external magnetic field, and by magnetically perturbed spectra of polycrystalline samples. The determination of the orientation of the electric field gradient tensor with respect to the molecular axes is a prerequisite for a decisive check of molecular orbital calculations on this type of binuclear bio-inorganic systems. (See also the preceding paper in this journal.)
    Type of Medium: Electronic Resource
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  • 29
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] Fig. 1 (upper part) shows the changes observed after addition of sodium and (lower part) after dialysis with a hypotonic solution of sodium chloride, as against 10 control animals (maternal serum: 136 ± 3-6 m.equiv./ 1., foetal serum: 146 ± 7-9 m.equiv./l., amniotic fluid: 136 ...
    Type of Medium: Electronic Resource
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  • 30
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Biomembranes 513 (1978), S. 244-254 
    ISSN: 0005-2736
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
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