<< Chapter < Page Chapter >> Page >

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

what does preconceived mean
sammie Reply
physiological Psychology
Nwosu Reply
How can I develope my cognitive domain
Amanyire Reply
why is communication effective
Dakolo Reply
Communication is effective because it allows individuals to share ideas, thoughts, and information with others.
effective communication can lead to improved outcomes in various settings, including personal relationships, business environments, and educational settings. By communicating effectively, individuals can negotiate effectively, solve problems collaboratively, and work towards common goals.
it starts up serve and return practice/assessments.it helps find voice talking therapy also assessments through relaxed conversation.
miss
Every time someone flushes a toilet in the apartment building, the person begins to jumb back automatically after hearing the flush, before the water temperature changes. Identify the types of learning, if it is classical conditioning identify the NS, UCS, CS and CR. If it is operant conditioning, identify the type of consequence positive reinforcement, negative reinforcement or punishment
Wekolamo Reply
please i need answer
Wekolamo
because it helps many people around the world to understand how to interact with other people and understand them well, for example at work (job).
Manix Reply
Agreed 👍 There are many parts of our brains and behaviors, we really need to get to know. Blessings for everyone and happy Sunday!
ARC
A child is a member of community not society elucidate ?
JESSY Reply
Isn't practices worldwide, be it psychology, be it science. isn't much just a false belief of control over something the mind cannot truly comprehend?
Simon Reply
compare and contrast skinner's perspective on personality development on freud
namakula Reply
Skinner skipped the whole unconscious phenomenon and rather emphasized on classical conditioning
war
explain how nature and nurture affect the development and later the productivity of an individual.
Amesalu Reply
nature is an hereditary factor while nurture is an environmental factor which constitute an individual personality. so if an individual's parent has a deviant behavior and was also brought up in an deviant environment, observation of the behavior and the inborn trait we make the individual deviant.
Samuel
I am taking this course because I am hoping that I could somehow learn more about my chosen field of interest and due to the fact that being a PsyD really ignites my passion as an individual the more I hope to learn about developing and literally explore the complexity of my critical thinking skills
Zyryn Reply
good👍
Jonathan
and having a good philosophy of the world is like a sandwich and a peanut butter 👍
Jonathan
generally amnesi how long yrs memory loss
Kelu Reply
interpersonal relationships
Abdulfatai Reply
What would be the best educational aid(s) for gifted kids/savants?
Heidi Reply
treat them normal, if they want help then give them. that will make everyone happy
Saurabh
What are the treatment for autism?
Magret Reply
hello. autism is a umbrella term. autistic kids have different disorder overlapping. for example. a kid may show symptoms of ADHD and also learning disabilities. before treatment please make sure the kid doesn't have physical disabilities like hearing..vision..speech problem. sometimes these
Jharna
continue.. sometimes due to these physical problems..the diagnosis may be misdiagnosed. treatment for autism. well it depends on the severity. since autistic kids have problems in communicating and adopting to the environment.. it's best to expose the child in situations where the child
Jharna
child interact with other kids under doc supervision. play therapy. speech therapy. Engaging in different activities that activate most parts of the brain.. like drawing..painting. matching color board game. string and beads game. the more you interact with the child the more effective
Jharna
results you'll get.. please consult a therapist to know what suits best on your child. and last as a parent. I know sometimes it's overwhelming to guide a special kid. but trust the process and be strong and patient as a parent.
Jharna
Got questions? Join the online conversation and get instant answers!
Jobilize.com Reply
Practice Key Terms 2

Get Jobilize Job Search Mobile App in your pocket Now!

Get it on Google Play Download on the App Store Now




Source:  OpenStax, Chemistry. OpenStax CNX. May 20, 2015 Download for free at http://legacy.cnx.org/content/col11760/1.9
Google Play and the Google Play logo are trademarks of Google Inc.

Notification Switch

Would you like to follow the 'Chemistry' conversation and receive update notifications?

Ask