Metabolism of Non Glucose Sugars

METABOLISM OF
NON GLUCOSE
SUGARS
DR: FESTO NGOWI
MD, RESIDENT OG
INTRODUCTION
Non Glucose sugars refers to hexose
sugars with similar chemical
composition to glucose but with
different chemical structure.
EXAMPLES;
Fructose
Galactose
Manose
OVERVIEW
Fructose Metabolism
Clinical Significances of Fructose Metabolism
Galactose Metabolism
Clinical Significances of Galactose Metabolism
Mannose Metabolism
Clinical Significances of Mannose Metabolism

FRUCTOSE METABOLISM
Fructose can be derived from sucrose
(disaccharide of glucose and fructose).
Diets containing large amounts of
sucrose can utilize the fructose as a
major source of energy.
The pathway to utilization of fructose
differs in muscle and liver.
Muscle which contains hexokinase
can phosphorylate fructose to F6P
which is a direct glycolytic
intermediate.
FRUCTOSE METABOLISM
CONT.
In the liver which contains mostly
glucokinase, which is specific for
glucose as its substrate, requires the
function of additional enzymes to
utilize fructose in glycolysis.
Hepatic fructose is phosphorylated
on C1 by fructokinase yielding
fructose-1-phosphate (F1P).
FRUCTOSE METABOLISM
CONT.
Fructose aldolase utilizes F1P as
substrates generates
dihydroxyacetone phosphate
(DHAP) and glyceraldehyde.
The DHAP is converted into
Glyceraldehyde-3-Phosphate by triose
phosphate isomerase, and enters
glycolysis.
The glyceraldehyde is phosphorylated
to G3P by glyceraldehyde kinase.
FRUCTOSE METABOLISM
CONT.
Clinical Significances of
Fructose Metabolism
Varios inherited abnormalities in fructose
metabolism have been identified.
Essential fructosuria is a benign
metabolic disorder caused by the lack of
fructokinase which is normally present in
the liver, pancreatic islets and kidney
cortex.
The fructosuria of this disease depends
on the time and amount of fructose and
sucrose intake.
Since the disorder is asymptomatic and
harmless it may go undiagnosed.
Fructose Metabolism
Hereditary fructose intolerance is a
potentially lethal disorder resulting from a
lack of aldolase B which is normally
present in the liver, small intestine and
kidney cortex.
Severe hypoglycemia result following
fructose uptake;
This is caused by fructose-1-phosphate
inhibition of glycogenolysis, by interfering
with the glycogen phosphorylase reaction,
and inhibition of gluconeogenesis at the
deficient aldolase step.
Fructose Metabolism
Prolonged intake of fructose by
infants with this defect leads to
reduced hepatic function,
hepatomegaly, and eventually
hepatic failure and death.
Patients remain symptom free on a
diet devoid of fructose and sucrose.
Fructose Metabolism
In weight reduction, Fructose is
recommended in place of glucose;
Why?
Fructose yields same amount of energy
(ATP),
But its much sweeter than Glucose,
So in the end you will consume small
ammount of sugar for the same level of
sweetness.
Galactose, is metabolized from the
milk sugar, lactose (a disaccharide of
glucose and galactose).
Enters glycolysis by its conversion to
glucose-1-phosphate (G1P).
This occurs through a series of
steps.
First the galactose is phosphorylated
by galactokinase to yield galactose-
1-phosphate.
Galactose Metabolism cont.
Epimerization of galactose-1-phosphate
to G1P requires an enzyme galactose-1-
phosphate uridyl transferase, which
transfer UDP from uridine
diphosphoglucose (UDP-glucose) to yield
UDP-galactose and Glucose-1-phosphate.
Glucose-1-phosphate which then is
converted to G6P by phosphoglucose
mutase.
Galactose Metabolism cont.
Three inherited disorders of galactose
metabolism have been studied.
Classic galactosemia is a major
symptom of two enzyme defects.
One results from deficiency of the enzyme
galactose-1-phosphate uridyl transferase.
The second form of galactosemia results
from a deficiency of galactokinase.
These two defects are manifest by failure
to thrive in neonates .
Clinical findings of these disorders
include;
Vomiting and diarrhea
Hypergalactosemia,
Urinary galactitol excretion
Elevated liver function
In a long term, these galactosemias can
go on to produce blindness and fatal liver
damage.
Blindness is due to the conversion of
circulating galactose to the sugar alcohol
galacitol, by a galactose reductase
(NADPH-dependent) that is present in
neural tissue and lens of the eye.
High concentration of galacitol in the
lens causes osmotic swelling, with the
resultant formation of cataracts.
The principal treatment of these
disorders is to eliminate lactose from the
diet.
Mannose Metabolism
Mannose is a C2 epimer of glucose.
The digestion of many polysaccharides
and glycoproteins yields mannose.
Manose is phosphorylated by hexokinase
to generate mannose-6-phosphate.
Mannose-6-phosphate is converted to
fructose-6-phosphate, by the enzyme
phosphomannose isomerase, and then
enters the glycolytic pathway or is
converted to glucose-6-phosphate by the
gluconeogenic pathway of hepatocytes.
Manose.
Mannose is not well metabolized in
humans.
Mannose have been detected in all
body tissues, using radioactive
markers, in a well hydrated mammal,
90% of mannose ingested is excreted
unchanched into the urine within 30 -
60 minutes, with 99% of the remainder
being excreted within the following 8
hours.
There is no significant increase in
blood-glucose levels during this time.
Manose.
D-mannose is claimed as a
naturopathic remedy for urinary
tract infections, and it is claimed to
work through the disruption of
adherence of bacteria in the urinary
tract (competition to binding sites).
THE END
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METABOLISM OF

Clinical Significances of Fructose Metabolism

Clinical Significances of Galactose Metabolism

Clinical Significances of Mannose Metabolism

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