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Metabolism of Other Hexoses
Group I ( Roll no. 1 – 50)
Presenters
Kyaw San Lin & KK Thwe Sunn
Kaung Kyaw Min & Kay Yu San
Slideshow preparation
Kyaw Khan Zaw & Kyaw San Lin
CONTENTS
Introduction
Fructose metabolism
Galactose metabolism
Mannose metabolism
Summary
References
INTRODUCTION
 Fate of dietary carbohydrates after digestion
& absorption – intermediary metabolism
 End products of carbohydrate digestion –
monosaccharides: hexoses and pentoses
 Principle hexoses – glucose, galactose &
fructose
 80 – 100% of the total amount absorbed
from GI tract - glucose
 Remaining 20% or less – mannose,
galactose & fructose
 After absorption, all enter the liver and
converted mainly to glucose.
SOURCES
 Disaccharide sucrose
 As free monosaccharide
METABOLISM
The entry of fructose into the cells is not insulin dependent.
DISORDERS OF FRUCTOSE METABOLISM
EXCESSIVE FRUCTOSE CONSUMPTION
Fructose ↑
Fructose 1-P ↑
Pi ↓
ATP ↓
Gluconeogenesis ↓
Hypoglycemia
Protein synthesis ↓
Clotting factors &
other essential
proteins ↓
AMP ↑
AMP degraded
Hyperuricaemia
Gout
DHAP ↑
Glycerol 3-P ↑
entry into
glycolysis ↑
Pyruvate ↑
Acetyl CoA ↑
Fatty acid ↑
Hypertriacyl-
glycerolaemia
Cholestrol ↑
Hypercholes-
trolemia
Atherogenic
ESSENTIAL FRUCTOSURIA
It is benign and asymptomatic.
HEREDITARY FRUCTOSE INTOLERANCE
(FRUCTOSE POISONING)
o Autosomal recessive (1:20,000 births)
o Symptoms similar to excessive fructose consumption.
o Sucrose & fructose must be removed from the diet.
FORMATION OF SORBITOL
D-Glucose
NADPH+
H
+
NADP
+
ALDOSE
REDUCTASE
(not found in liver)
D-Sorbitol
SORBITOL
DEHYDROGENASE
NAD+
NADH+H
+
D-Fructose
FORMATION OF SORBITOL
 Increases in activity as the glucose concentration
rises
 In tissues that are not insulin-sensitive
 Fructose and sorbitol in
 Lens → Cataract
 Peripheral nerves → Neuropathy
 Renal glomeruli → Nephropathy
SOURCE
 lactose obtained from milk and its products
METABOLISM
in liver
Enters
glycolysis
in
liver
LACTATING MOTHERS
In lactating
mammary
gland
If Gal in diet is
insufficient
DISORDER OF GALACTOSE METABOLISM
In lactating
mammary
glandin liver
Enters
glycolysis
If Gal in diet is
insufficient
in
liver
GALACTOSEMIA
 Is a severe disease that is inhirited as an
autosomal recessive trait ( 1 in 35,000 birth)
 Physiologic consequences are similar to
those found in hereditary fructose
intolerance
 A broader spectrum of tissues is affected.
 Vomiting diarrhea occurs and enlarge liver
with jaundice is common
GALACTOSEMIA
 The accumulated galactose is shunted into
side pathways such as that of galactitol
production.
 Accumulation of galactitol occurs in
 lens of the eye → cataract
 Liver → liver damage
 Nerve → mental retardation
SOURCES
 Vegetables
 Hydrolysis of mannan gum
 There is little mannose in dietary
carbohydrates
METABOLISM
SUMMARY
Glucos
e
Glucose 6-P
Fructose 6-P
Fructose 1,6-bisphosphate
Dihydroxyacetone
phosphate
Glyceraldehyde 3-PFructose
Fructose 1-P
Galactose
Galactose 1-P
Glucose 1-P
Mannose
Mannose 6-P
Glyceraldehyde
glycolysis
REFERENCES
 Department of Biochemistry: Biochemistry Handbook
Volume 3. University of Medicine Mandalay, 2012.
 Murray RK, Bender DA, et al: Harper’s Illustrated
Biochemistry, 28th ed. McGraw Hill, 2009.
 Harvey R, Ferrier D: Lippincott’s Illustrated Reviews:
Biochemistry, 5th ed. Lippincott Williams & Wilkins, 2011.
 Bray GA, Nielsen SJ, Popkin BM: Consumption of high-
fructose corn syrup in beverages may play a role in the
epidemic of obesity. Am J Clin Nutr 2004;79:537–43.
 Glyconutrition ForLife
http://www.glyconutritionforlife.org/Science_of_Glyconutri
ents/Mannose.php accessed at 8 p.m, 23/7/2012.
 Medical and healthcare design slides - Templates -
Office.com http://office.microsoft.com/en-
us/templates/CT010238352.aspx accessed at
8:27p.m.,13/7/2012
THANK YOU!

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Metabolism of Other Hexoses

  • 1. Metabolism of Other Hexoses Group I ( Roll no. 1 – 50) Presenters Kyaw San Lin & KK Thwe Sunn Kaung Kyaw Min & Kay Yu San Slideshow preparation Kyaw Khan Zaw & Kyaw San Lin
  • 4.  Fate of dietary carbohydrates after digestion & absorption – intermediary metabolism  End products of carbohydrate digestion – monosaccharides: hexoses and pentoses  Principle hexoses – glucose, galactose & fructose  80 – 100% of the total amount absorbed from GI tract - glucose  Remaining 20% or less – mannose, galactose & fructose  After absorption, all enter the liver and converted mainly to glucose.
  • 5.
  • 6. SOURCES  Disaccharide sucrose  As free monosaccharide
  • 7. METABOLISM The entry of fructose into the cells is not insulin dependent.
  • 8.
  • 9.
  • 10.
  • 11. DISORDERS OF FRUCTOSE METABOLISM
  • 12. EXCESSIVE FRUCTOSE CONSUMPTION Fructose ↑ Fructose 1-P ↑ Pi ↓ ATP ↓ Gluconeogenesis ↓ Hypoglycemia Protein synthesis ↓ Clotting factors & other essential proteins ↓ AMP ↑ AMP degraded Hyperuricaemia Gout DHAP ↑ Glycerol 3-P ↑ entry into glycolysis ↑ Pyruvate ↑ Acetyl CoA ↑ Fatty acid ↑ Hypertriacyl- glycerolaemia Cholestrol ↑ Hypercholes- trolemia Atherogenic
  • 13. ESSENTIAL FRUCTOSURIA It is benign and asymptomatic.
  • 14. HEREDITARY FRUCTOSE INTOLERANCE (FRUCTOSE POISONING) o Autosomal recessive (1:20,000 births) o Symptoms similar to excessive fructose consumption. o Sucrose & fructose must be removed from the diet.
  • 15. FORMATION OF SORBITOL D-Glucose NADPH+ H + NADP + ALDOSE REDUCTASE (not found in liver) D-Sorbitol SORBITOL DEHYDROGENASE NAD+ NADH+H + D-Fructose
  • 16. FORMATION OF SORBITOL  Increases in activity as the glucose concentration rises  In tissues that are not insulin-sensitive  Fructose and sorbitol in  Lens → Cataract  Peripheral nerves → Neuropathy  Renal glomeruli → Nephropathy
  • 17.
  • 18. SOURCE  lactose obtained from milk and its products
  • 20. LACTATING MOTHERS In lactating mammary gland If Gal in diet is insufficient
  • 21. DISORDER OF GALACTOSE METABOLISM In lactating mammary glandin liver Enters glycolysis If Gal in diet is insufficient in liver
  • 22. GALACTOSEMIA  Is a severe disease that is inhirited as an autosomal recessive trait ( 1 in 35,000 birth)  Physiologic consequences are similar to those found in hereditary fructose intolerance  A broader spectrum of tissues is affected.  Vomiting diarrhea occurs and enlarge liver with jaundice is common
  • 23. GALACTOSEMIA  The accumulated galactose is shunted into side pathways such as that of galactitol production.  Accumulation of galactitol occurs in  lens of the eye → cataract  Liver → liver damage  Nerve → mental retardation
  • 24.
  • 25. SOURCES  Vegetables  Hydrolysis of mannan gum  There is little mannose in dietary carbohydrates
  • 27. SUMMARY Glucos e Glucose 6-P Fructose 6-P Fructose 1,6-bisphosphate Dihydroxyacetone phosphate Glyceraldehyde 3-PFructose Fructose 1-P Galactose Galactose 1-P Glucose 1-P Mannose Mannose 6-P Glyceraldehyde glycolysis
  • 28. REFERENCES  Department of Biochemistry: Biochemistry Handbook Volume 3. University of Medicine Mandalay, 2012.  Murray RK, Bender DA, et al: Harper’s Illustrated Biochemistry, 28th ed. McGraw Hill, 2009.  Harvey R, Ferrier D: Lippincott’s Illustrated Reviews: Biochemistry, 5th ed. Lippincott Williams & Wilkins, 2011.  Bray GA, Nielsen SJ, Popkin BM: Consumption of high- fructose corn syrup in beverages may play a role in the epidemic of obesity. Am J Clin Nutr 2004;79:537–43.  Glyconutrition ForLife http://www.glyconutritionforlife.org/Science_of_Glyconutri ents/Mannose.php accessed at 8 p.m, 23/7/2012.  Medical and healthcare design slides - Templates - Office.com http://office.microsoft.com/en- us/templates/CT010238352.aspx accessed at 8:27p.m.,13/7/2012

Editor's Notes

  1. First symptoms appear when a baby is weaned from milk and begins to be fed food containing sucrose or fructose. Some of these symptoms may also be responsible for obesity resulting from high fructose diet.
  2. Nephropathy picture
  3. UDP-galactose serves as the donor of galactose units in a number of synthetic pathways, including synthesis of lactose, glycoproteins, glycolipids, and proteoglycans.
  4. The word “mannose” comes from the word ‘Manna”, the foo that was eaten by the Israelites during their wandering through the desert. It is refer to the secretion of tamarisk tree that was plentiful in the Sinai Peninsula during those days. Its resin or wax was sweet to taste and yellow in colour.