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Definition
are proteins that contain many chains of glycosaminoglycans, which are long
unbranched polysaccharides composed of repeating disaccharide units. Good lubricants, important in joints |
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Term
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Definition
shorter carbohydrate chains than proteoglycans. Most blood proteins (hormones, antibodies,
enzymes) are glycoproteins. O-linked or N-linked. Cell surface proteins. |
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Term
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Definition
lysosomal storage disease. lack the phosphotransferase that is required for formation of M6P
(mannose 6-phosphate) residues on lysosomal enzymes in the cis-Golgi. |
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Definition
| deficiency in hexosaminidase A; mental retardation, blindness, death between 2nd and 3rd years |
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Term
| What kind of carbohydrate linkages can humans not digest? |
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Definition
| alpha-galactosidic or beta-glucosidic |
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Term
| What is the only coenzyme required for the pentose phosphate pathway, and what does it participate in? |
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Definition
The only coenzyme required for PPP is thiamine pyrophosphate (TTP) by
transketolase. The transketolase activity in RBCs is used to measure thiamine nutritional status and diagnose the presence of thiamine deficiency. (Remember that TTP was also required by pyruvate dehydrogenase complex and a-ketoglutarate dehydrogenase- |
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Term
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Definition
| Two types: Fructokinase defiency is harmless and results in fructose being excreted in the urine. Aldolase b defiency is serious, it results in fructose being phosphorylated but not going any further. F-1-P builds up in the liver leading to lactic acidosis and hypoglycemia due to it stopping gluconeogenesis and glycogenolysis. Severe Liver damage occurs. |
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Definition
| converts glucose to fructose. important in seminal vesicles |
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Term
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Definition
Specific reactions catalyzed by this enzyme include:
glucose + NADPH + H+--> sorbitol + NADP+ ; and
galactose + NADPH + H+--> galactitol + NADP+ |
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Term
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Definition
caused by a defect in galactose 1-phosphate uridyltransferase; galactose 1-P accumulates in tissues and
galactose is elevated in blood and urine leading to high galacticol levels. |
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Term
| What inhibits Glucose 6-phosphate dehydrogenase |
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Definition
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Term
| What enzyme catalyzes the rate limiting step in fructose metabolism |
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Definition
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Term
| What enzyme is deficient in Non-classical galactosemia |
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Definition
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