Galactose 1‐phosphate accumulates to high levels in galactose‐treated cells due to low GALT activity and absence of product inhibition of GALK

2019 ◽  
Vol 43 (3) ◽  
pp. 529-539 ◽  
Author(s):  
Sher Li Oh ◽  
Li Yi Cheng ◽  
Jie Fu J Zhou ◽  
Wolfgang Henke ◽  
Thilo Hagen
Keyword(s):  
Product Inhibition ◽  
Galt Activity

scholarly journals Qualitative Properties of Autocatalytic Reactions Occurring in a Flow System

1985 ◽  
Vol 40 (7) ◽  
pp. 736-747
Author(s):  
Sang H. Kim ◽  
Vladimir Hlavacek
Keyword(s):  
Chaotic Behavior ◽  
Flow System ◽  
Cell Model ◽  
Dispersion Model ◽  
Travelling Waves ◽  
Continuous Model ◽  
Product Inhibition ◽  
Stable Node ◽  
Qualitative Properties ◽  
Intermediate Value

The dynamic behavior of an autocatalytic reaction with a product inhibition term is studied in a flow system. A unique steady state exists in the continuous tank reactor. Linear stability analysis predicts either a stable node, a focus or an unstable saddle-focus. Sustained oscillations around the unstable focus can occur for high values of the Damköhler number (Da). In the distributed system, travelling, standing or complex oscillatory waves are detected. For a low value of Da, travelling waves with a pseudo-constant pattern are observed. With an intermediate value of Da, single or multiple standing waves are obtained. The temporal behavior indicates also the appearance of retriggering or echo waves. For a high value of Da, both single peak and complex multipeak oscillations are found. In the cell model, both regular oscillations near the inlet and chaotic behavior downstream are observed. In the dispersion model, higher Peclet numbers (Pe) eliminate the oscillations. The spatial profile shows a train of pulsating waves for the discrete model and a single pulsating or solitary wave for the continuous model.

scholarly journals The Mechanism of End Product Inhibition of Serine Biosynthesis

1974 ◽  
Vol 249 (5) ◽  
pp. 1348-1355
Author(s):  
Ilga Winicov ◽  
Lewis I. Pizer
Keyword(s):  
Product Inhibition ◽  
Serine Biosynthesis

scholarly journals Current and Future Treatments for Classic Galactosemia

2021 ◽  
Vol 11 (2) ◽  
pp. 75 ◽  
Author(s):  
Britt Delnoy ◽  
Ana I. Coelho ◽  
Maria Estela Rubio-Gozalbo
Keyword(s):  
Standard Of Care ◽  
Type I ◽  
Therapeutic Approaches ◽  
Restricted Diet ◽  
Galactose Metabolism ◽  
Galt Activity ◽  
Classic Galactosemia ◽  
Pathogenic Factors ◽  
Future Treatments ◽  
Novel Therapeutic

Type I (classic) galactosemia, galactose 1-phosphate uridylyltransferase (GALT)-deficiency is a hereditary disorder of galactose metabolism. The current therapeutic standard of care, a galactose-restricted diet, is effective in treating neonatal complications but is inadequate in preventing burdensome complications. The development of several animal models of classic galactosemia that (partly) mimic the biochemical and clinical phenotypes and the resolution of the crystal structure of GALT have provided important insights; however, precise pathophysiology remains to be elucidated. Novel therapeutic approaches currently being explored focus on several of the pathogenic factors that have been described, aiming to (i) restore GALT activity, (ii) influence the cascade of events and (iii) address the clinical picture. This review attempts to provide an overview on the latest advancements in therapy approaches.

scholarly journals Product inhibition studies on bovine liver carbamoyl phosphate synthetase

1974 ◽  
Vol 141 (3) ◽  
pp. 817-824 ◽  
Author(s):  
Keith R. F. Elliott ◽  
Keith F. Tipton
Keyword(s):  
Inorganic Phosphate ◽  
Substrate Binding ◽  
Inorganic Ions ◽  
Bovine Liver ◽  
Product Inhibition ◽  
Carbamoyl Phosphate Synthetase ◽  
Carbamoyl Phosphate ◽  
Product Release ◽  
Proposed Model ◽  
Inhibition Studies

A study of the product-inhibition patterns of carbamoyl phosphate synthetase from bovine liver is reported. Inhibition by adenosine, AMP and inorganic ions is also reported. The results are in agreement with the previously proposed model in which the order of substrate binding is ATPMg, followed by HCO3−, ATPMg and NH4+. The order of product release on the basis of the reported results is carbamoyl phosphate, followed by ADPMg, ADPMg and inorganic phosphate.

Structure-activity relations and β-lactamase resistance

1980 ◽  
Vol 289 (1036) ◽  
pp. 197-205 ◽  
Keyword(s):  
Broad Spectrum ◽  
Product Inhibition ◽  
Sulphur Atom ◽  
Chemical Modifications ◽  
Gram Negative ◽  
Structure Activity ◽  
Broad Spectrum Resistance ◽  
Structure Activity Relations ◽  
Minor Influence ◽  
A Minor

β-Lactam antibiotics resistant to β-lactamase degradation can be produced by many chemical modifications, but often at the expense of antibacterial activity. Substitution onto several positions in the molecule produces different and often selective resistance; for instance, heavily sterically hindered acyl groups give staphylococcal P-lactamase resistance to penicillins, and resistance to some enzymes from Gram-negative pathogens to both penicillins and cephalosporins. 6-α- or 7-α-substituents respectively confer a broad spectrum of resistance (e.g. cefoxitin), but changes at positions 2 or 3 have only a minor influence on enzyme susceptibility. Changes in the ring condensed with the β-lactam, such as changing ceph-3-em to ceph-2-em may greatly enhance stability. Small improvements can occur when the nuclear sulphur atom is oxidized, but a much better effect is obtained when it is replaced by another atom such as oxygen, as in clavulanic acid. This compound appears to have broad spectrum resistance which is actually due to susceptibility and subsequent product inhibition.

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