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Glucose-6-phosphate dehydrogenase deficiency

Enzymes in the human body act as catalysts for important chemical reactions in cellular metabolism. As such, a deficiency of a particular enzyme can translate to a life-threatening disease. G6PD (glucose-6-phosphate dehydrogenase) deficiency, a genetic condition that results in a shortage of the enzyme glucose-6-phosphate dehydrogenase, is the most common enzyme deficiency in humans. This enzyme, shown in [link] , is the rate-limiting enzyme for the metabolic pathway that supplies NADPH to cells ( [link] ).

A colorful model of the Glucose-6-phosphate dehydrogenase structure is shown. The molecule has two distinct lobes which are filled with spiraled ribbon-like regions of yellow, lavender, blue, silver, green, and pink.
Glucose-6-phosphate dehydrogenase is a rate-limiting enzyme for the metabolic pathway that supplies NADPH to cells.

A disruption in this pathway can lead to reduced glutathione in red blood cells; once all glutathione is consumed, enzymes and other proteins such as hemoglobin are susceptible to damage. For example, hemoglobin can be metabolized to bilirubin, which leads to jaundice, a condition that can become severe. People who suffer from G6PD deficiency must avoid certain foods and medicines containing chemicals that can trigger damage their glutathione-deficient red blood cells.

A reaction mechanism is diagrammed in this figure. At the left, the name Glucose is followed by a horizontal, right pointing arrow, labeled, “Hexokinase.” Below this arrow and to the left is a yellow star shape labeled, “A T P.” A curved arrow extends from this shape to the right pointing arrow, and down to the right to a small brown oval labeled, “A D P.” To the right of the horizontal arrow is the name Glucose 6 phosphate, which is followed by another horizontal, right pointing arrow which is labeled, “G 6 P D.” A small orange rectangle below and left of this arrow is labeled “N A D P superscript plus.” A curved arrow extends from this shape to the right pointing arrow, and down to the right to a small salmon-colored rectangle labeled “N A P D H.” A curved arrow extends from this shape below and to the left, back to the orange rectangle labeled, “N A D P superscript plus.” Another curved arrow extends from a green oval labeled “G S S G” below the orange rectangle, up to the arrow curving back to the orange rectangle. This last curved arrow continues on to the lower right to a second green oval labeled, “G S H.” The end of this curved arrow is labeled, “Glutathione reductase.” To the right of the rightmost horizontal arrow appears the name 6 phosphogluconate.
In the mechanism for the pentose phosphate pathway, G6PD catalyzes the reaction that regulates NAPDH, a co-enzyme that regulates glutathione, an antioxidant that protects red blood cells and other cells from oxidative damage.

Heterogeneous catalysts

A heterogeneous catalyst    is a catalyst that is present in a different phase (usually a solid) than the reactants. Such catalysts generally function by furnishing an active surface upon which a reaction can occur. Gas and liquid phase reactions catalyzed by heterogeneous catalysts occur on the surface of the catalyst rather than within the gas or liquid phase.

Heterogeneous catalysis has at least four steps:

  1. Adsorption of the reactant onto the surface of the catalyst
  2. Activation of the adsorbed reactant
  3. Reaction of the adsorbed reactant
  4. Diffusion of the product from the surface into the gas or liquid phase (desorption).

Any one of these steps may be slow and thus may serve as the rate determining step. In general, however, in the presence of the catalyst, the overall rate of the reaction is faster than it would be if the reactants were in the gas or liquid phase.

[link] illustrates the steps that chemists believe to occur in the reaction of compounds containing a carbon–carbon double bond with hydrogen on a nickel catalyst. Nickel is the catalyst used in the hydrogenation of polyunsaturated fats and oils (which contain several carbon–carbon double bonds) to produce saturated fats and oils (which contain only carbon–carbon single bonds).

In this figure, four diagrams labeled a through d are shown. In each, a green square surface is shown in perspective to provide a three-dimensional appearance. In a, the label “N i surface” is placed above with a line segment extending to the green square. At the lower left and upper right, pairs of white spheres bonded tougher together appear as well as white spheres on the green surface. Black arrows are drawn from each of the white spheres above the surface to the white sphere on the green surface. In b, the white spheres are still present on the green surface. Near the center of this surface is a molecule with two central black spheres with a double bond indicated by two horizontal black rods between them. Above and below to the left and right, a total of four white spheres are connected to the black spheres with white rods. A line segment extends from this structure to the label, “Ethylene absorbed on surface breaking pi bonds.” Just above this is a nearly identical structure greyed out with three downward pointing arrows to the black and white structure to indicate downward motion. The label “Ethylene” at the top of the diagram is connected to the greyed out structure with a line segment. In c, the diagram is very similar to b except that the greyed out structure and labels are gone and one of the white spheres near the black and white structure in each pair on the green surface is greyed out. Arrows point from the greyed out white spheres to the double bond between the two black spheres. In d, only a single white sphere remains from each pair in the green surface. A curved arrow points from the middle of the green surface to a model above with two central black spheres with a single black rod indicating a single bond between them. Each of the black rods has three small white spheres bonded as indicated by white rods between the black spheres and the small white spheres. The four bonds around each black sphere are evenly distributed about the black spheres.
There are four steps in the catalysis of the reaction C 2 H 4 + H 2 C 2 H 6 by nickel. (a) Hydrogen is adsorbed on the surface, breaking the H–H bonds and forming Ni–H bonds. (b) Ethylene is adsorbed on the surface, breaking the π-bond and forming Ni–C bonds. (c) Atoms diffuse across the surface and form new C–H bonds when they collide. (d) C 2 H 6 molecules escape from the nickel surface, since they are not strongly attracted to nickel.

Questions & Answers

Ayele, K., 2003. Introductory Economics, 3rd ed., Addis Ababa.
Widad Reply
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Ariel
?
Ariel
What is economics
Widad Reply
the study of how humans make choices under conditions of scarcity
AI-Robot
U(x,y) = (x×y)1/2 find mu of x for y
Desalegn Reply
U(x,y) = (x×y)1/2 find mu of x for y
Desalegn
what is ecnomics
Jan Reply
this is the study of how the society manages it's scarce resources
Belonwu
what is macroeconomic
John Reply
macroeconomic is the branch of economics which studies actions, scale, activities and behaviour of the aggregate economy as a whole.
husaini
etc
husaini
difference between firm and industry
husaini Reply
what's the difference between a firm and an industry
Abdul
firm is the unit which transform inputs to output where as industry contain combination of firms with similar production 😅😅
Abdulraufu
Suppose the demand function that a firm faces shifted from Qd  120 3P to Qd  90  3P and the supply function has shifted from QS  20  2P to QS 10  2P . a) Find the effect of this change on price and quantity. b) Which of the changes in demand and supply is higher?
Toofiq Reply
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innocent Reply
factors influencing supply
Petrus Reply
what is economic.
Milan Reply
scares means__________________ends resources. unlimited
Jan
economics is a science that studies human behaviour as a relationship b/w ends and scares means which have alternative uses
Jan
calculate the profit maximizing for demand and supply
Zarshad Reply
Why qualify 28 supplies
Milan
what are explicit costs
Nomsa Reply
out-of-pocket costs for a firm, for example, payments for wages and salaries, rent, or materials
AI-Robot
concepts of supply in microeconomics
David Reply
economic overview notes
Amahle Reply
identify a demand and a supply curve
Salome Reply
i don't know
Parul
there's a difference
Aryan
Demand curve shows that how supply and others conditions affect on demand of a particular thing and what percent demand increase whith increase of supply of goods
Israr
Hi Sir please how do u calculate Cross elastic demand and income elastic demand?
Abari
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Source:  OpenStax, Chemistry. OpenStax CNX. May 20, 2015 Download for free at http://legacy.cnx.org/content/col11760/1.9
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