HMP Shuntg6pd

Hexose Monophosphate Shunt
1. The Hexose Monophosphate Shunt is also known as Pentose phosphate Pathway

(PPP) or Phosphogluconate pathway.
2. This is an alternative Glucose oxidation pathway.
3. The pentose phosphate pathway takes place in the Cytosol.
4. Its primary role is anabolic rather than catabolic.
5. Biosynthesis of NADPH and Pentoses.
6. Steroidogenic tissues, red blood cells and liver are the major sites of hexose
monophosphate pathway.
7. The pentose phosphate pathway divided into two phases:
A. Oxidative phase: Each molecule of Glucose-6-Phosphate oxidized to Ribulose-
5-Phosphate. It generates NADPH with the release of CO2.
B. Non-oxidative phase: Catalyzes the inter-conversion of 3, 4, 5 and 7- carbon
sugars. Synthesize pentose-phosphate and other phosphate monosaccharides.
Oxidative Stage of Pentose Phosphate Pathway
Glucose-6-phosphate
NADP+
Glucose-6-phosphate
NADPH dehydrogenase
6-Phosphogluconolactone
H2O
Gluconolactonase
H+
6-Phosphogluconate
NADP+
6-Phosphogluconate
NADPH dehydrogenase
CO2
Ribulose-5-phosphate
Non-oxidative reactions
Non-Oxidative Stage of Pentose Phosphate Pathway
Ribulose-5-phosphate
Ribulose-5-phosphate 3-epimerase Ribulose-5-phosphate isomerase
Xylulose-5-phosphate Ribose-5-phosphate
Transketolase
Sedoheptulose-7-phosphate Glyceraldehyde-3-phosphate
Transaldolase
Erythrose-4-phosphate Fructose-6-phosphate
Transketolase
Glyceraldehyde-3-phosphate Fructose-6-phosphate
Significance of HMP Shunt

1. It is a source of NADPH and ribose-5-Phosphate.
2. The NADPH is required for the biosynthesis of fatty acid an steroids, drug reduction
(detoxification of drugs and foreign compounds), synthesis of certain amino acids
involving the enzyme glutamate dehydrogenase, phagocytosis and as a cofactor
for some non-synthetic enzymatic reactions.
3. The Ribose-5- Phosphate required for nucleic acid biosynthesis (RNA and DNA).
4. NADPH involved in Antioxidant (Free radical scavenging) reaction. Gltathione
mediated reduction of H2O2.
5. High concentration of NADPH in lens of eye is necessary to preserve the
transparency of the lens.
6. In the Hexose MonoPhosphate Shunt Pathway, few molecules of Glycolytic
intermediates (Glyceraldehyde-3-Phosphate and Fructose-6-Phosphate) are produced
these are directly involves in Glycolysis.
7. NADPH is also required to preserve the integrity of RBC membrane.
Trick to Remember Non Oxidative Pathway:
1. Glyceraldehyde-3- phosphate (3C)

2. Erythrose -4-phosphate (4C)
3. Xylulose-5- phosphate (5C’)
4. Ribose-5- phosphate (5C)
5. Fructose-6- phosphate (6C) Ribulose-5-Phosphate
6. Sedoheptulose-7- phosphate (7C)
(5C’): (5C) 5C’ 5C
(7C): (3C)
(4C): (6C) 7C 3C
(5C’): (4C)
(3C): (6C) 4C 6C
3C 6C
GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY (G6PD)
Definition: An X-linked genetic enzyme deficiency resulting in abnormal metabolism in red

blood cell and can cause haemolysis usually after exposure to certain medications, foods, or
even infections.
G6PD deficiency is a genetic abnormality that results in an inadequate amount of glucose-6-
phosphate dehydrogenase (G6PD) in the blood. It is inherited as an X-linked recessive
disorder. Glucose-6-phosphate dehydrogenase or G6PD, helps red blood cells (RBCs)
function normally. This deficiency can cause hemolytic anemia, usually after exposure to
certain medications, foods, or even infections.
Pathophysiology
Normally red cells are protected from the action of free radicals by glutathione. NADPH
produced in erythrocytes by pentose phosphate pathway is used to keep the glutathione in
reduced state, reduced glutathione (GSH) which is essentially required to:
1. Preserve the integrity of RBC membrane.
2. Keep the ferrous ion (Fe2+) of hemoglobin in the reduced state so that accumulation of
methemoglobin (Fe3+) is prevented, because accumulation of methhemoglobin and
peroxides in erythrocytes leading to hemolysis.
3. G6PD also protects red blood cells from potentially harmful byproducts, certain
medications or an infection.
Figure 1: Pentose Phosphate pathway
HMP shunt is only means of Deficiency of G6PD impairs the synthesis

providing NADPH in the of NADPH in RBC which maintain the
erythrocytes. reduced form of glutathione which is
essential for RBC
G6PD deficiency causes breakdown of

red blood cells, when the body is exposed
to certain drugs or the stress of infection.
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Figure 2: G6PD deficiency
*
ROS: Reactive oxygen species
Causes
The defective gene that causes G6PD deficiency is on the X chromosome. Men have only one
X chromosome, while women have two X chromosomes. In males, one altered copy of the
gene is enough to cause G6PD deficiency. In females, however, a mutation would have to
occur in both copies of the gene. Since it is unlikely for females to have two altered copies of
this gene, males are affected by G6PD deficiency much more frequently than females. In
people with G6PD deficiency, hemolytic anemia can occur after eating fava beans or certain
legumes. It may also be triggered by infections or by certain drugs, such as antimalarials,
sulfonamides, a medication used for treating various infections, aspirin, some nonsteroidal
anti-inflammatory medications (NSAIDs).
Symptoms
In more serious cases, a child may exhibit symptoms of hemolytic anemia (also known as a
hemolytic crisis), including, rapid heart rate, shortness of breath, fever, fatigue, dizziness,
paleness, jaundice, or yellowing of the skin and eyes particularly in new born, paleness (in
darker-skinned kids, paleness is sometimes best seen in the mouth, especially on the lips or
tongue), dark, tea-colored urine.
Diagnosis
1. Complete blood count and reticulocyte count.
2. In active G6PD deficiency, Heinz bodies can be seen in red blood cells on a blood
film.
3. Liver enzymes (to exclude other causes of jaundice).
4. Lactate dehydrogenase (elevated in hemolysis and a marker of hemolytic severity)
Haptoglobin (decreased in hemolysis).
5. A direct antiglobulin test (Coombs' test)
6. Beutler fluorescent spot test visually identifies NADPH produced by G6PD
under ultraviolet light. When the blood spot does not fluoresce, the test is positive.
Treatment
Treatment for G6PD deficiency consists of removing the trigger that is causing symptoms. If
the condition was triggered by an infection, then the underlying infection is treated
accordingly. Medications that may be destroying red blood cells are also discontinued. Once
G6PD deficiency has progressed to hemolytic anemia, treatment usually includes oxygen
therapy and a blood transfusion to replenish oxygen and red blood cell levels.

HMP Shuntg6pd

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Hexose Monophosphate Shunt

1. The Hexose Monophosphate Shunt is also known as Pentose phosphate Pathway

Oxidative Stage of Pentose Phosphate Pathway

Significance of HMP Shunt

Trick to Remember Non Oxidative Pathway:

1. Glyceraldehyde-3- phosphate (3C)

Definition: An X-linked genetic enzyme deficiency resulting in abnormal metabolism in red

Figure 1: Pentose Phosphate pathway

HMP shunt is only means of Deficiency of G6PD impairs the synthesis

G6PD deficiency causes breakdown of

Figure 2: G6PD deficiency

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