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Monosaccharide derivatives
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1. Monosaccharide derivatives are important metabolic products, although excesses or deficiencies of some contribute to pathogenic conditions. 2. Sugar acids a. Ascorbic acid (vitamin C) is required in the synthesis of collagen. Scurvy: vitamin C deficiency produces abnormal collagen. (1) Prolonged deficiency of vitamin C causes scurvy (i.e., perifollicular petechiae, corkscrew hairs, bruising, gingival inflammation, and bleeding). b. Glucuronic acid reacts with bilirubin in the liver, forming conjugated (direct) bilirubin, which is water soluble.

Glucuronic acid: reacts with bilirubin to produce conjugated bilirubin c. Glucuronic acid is a component of glycosaminoglycans (GAGs), which are major constituents of the extracellular matrix. 3. Deoxy sugars a. 2-Deoxyribose is an essential component of the deoxyribonucleotide structure. 2-Deoxyribose: component of deoxyribonucleotide structure 4. Sugar alcohols (polyols) a. Glycerol derived from hydrolysis of triacylglycerol is phosphorylated in the liver to form glycerol phosphate, which enters the gluconeogenic pathway. (1) Liver is the only tissue with glycerol kinase to phosphorylate glycerol. Glycerol 3-phosphate: substrate for gluconeogenesis and for synthesizing triacylglycerol b. Sorbitol derived from glucose is osmotically active and is responsible for damage to the lens (cataract formation), Schwann cells (peripheral neuropathy), and pericytes (retinopathy), all associated with diabetes mellitus. c. Galactitol derived from galactose contributes to cataract formation in galactosemia.

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Sorbitol: cataracts, neuropathy, and retinopathy in diabetes mellitus 5. Amino sugars a. Replacement of the hydroxyl group with an amino group yields glucosamine and galactosamine. b. N-acetylated forms of these compounds are present in GAGs. 6. Sugar esters a. Sugar forms glycosidic bonds with phosphate or sulfate. b. Phosphorylation of glucose after it enters cells effectively traps it as glucose-6-phosphate, which is further metabolized. Phosphorylation of glucose: traps it in cells for further metabolism 7. Glycosylation Glycosylation of basement membranes of small vessels renders them permeable to proteins. a. Refers to the reaction of sugar aldehyde with protein amino groups to form a nonreversible covalent bond. b. Excessive glycosylation in diabetes leads to endothelial membrane alteration, producing microvascular disease. c. In arterioles, glycosylation of the basement membrane renders them permeable to protein, producing hyaline arteriolosclerosis. Hemoglobin A : formed by glucose reaction with
1c

terminal amino groups and used clinically as a measure of long-term blood glucose concentration Table 1-1. Monosaccharides Common in Metabolic Processes CARBONYL CLASS/SUGAR* GROUP MAJOR METABOLIC ROLE Triose (3 Carbons) Glyceraldehyde Aldose Intermediate in glycolytic and pentose phosphate pathways Dihydroxyacetone Ketose Reduced to glycerol (used in fat metabolism); present in glycolytic pathway Tetrose (4 Carbons) Erythrose Aldose Intermediate in pentose

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phosphate pathway Pentose (5 Carbons) Ribose Aldose Ribulose Ketose Component of RNA; precursor of DNA Intermediate in pentose phosphate pathway Absorbed from intestine with Na and enters cells; starting point of glycolytic pathway; polymerized to form glycogen in liver and muscle Absorbed from intestine by facilitated diffusion and enters cells; converted to intermediates in glycolytic pathway; derived from sucrose Absorbed from intestine with Na and enters cells; converted to glucose; derived from lactose Intermediate in pentose phosphate pathway
+ +

Hexose (6 Carbons) Glucose Aldose

Fructose

Ketose

Galactose

Aldose

Heptose (7 Carbons) Sedoheptulose Ketose

*Within cells, sugars usually are phosphorylated, which prevents them from diffusing out of the cell.

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