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1.4
| Question | Answer |
|---|---|
| deficiency of ADOLASE B -Fru-1-P accumulation in liver -hypoglycemia -liver damage(jaundice, hepatomegaly) -inhib of glycogenolysis & gluconeogenosis | Hereditary Fructose Intolerance |
| deficiency of FRUCTOKINASE -fructose accumulation -increased fructose in blood -fructose in urine | Essential Fructosuria |
| deficiency of GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE -hypoglycemia in fasting state -jaundice, hepatomegaly, cataracts, mental retardation | Classic Galactosemia |
| deficiency of GALACTOKINASE -galactosemia, galactosuria, galactitol accumulation -cataract formation | Non-Classic Galactosemia |
| -binds GALACTOSE to free GLUCOSE -found only in lactating mammary glands -lowers Km of galactosyl transferase, allowing for lactose synthesis | a-lactalbumin |
| transfers galactose and binds to membrane glucose (acetyllactosamine) forming B-1,4 bond | galactosyl transferase |
| amylose bond | a-1,4 linear |
| amylopectin bond | a-1,4 linear & a-1,6 branched |
| glycogen bond | a-1,4 linear & a-1,6 branched |
| cellulose bond | B-D-1,4 linear |
| sucrose bond | a,B-1,2 stacked |
| lactose bond | B-1,4 linear |
| trehalose bond | aa-1,1 linear |
| maltose bond | a-1,4 linear |
| maltotriose bond | a-1,4 linear |
| a-dextrin bond | a-1,4 linear & a-1,6 branched |
| isomaltose bond | a-1,6 stacked |
| raffinose bond | 1,3 linear & a,6 stacked |
| cellobiose bond | B-1,4 |
| celloboise monomer | glucose |
| raffinose monomer | galactose, fructose, glucose (galactose + sucrose) |
| isomaltose monomer | glucose |
| a-dextrin monomer | glucose |
| maltotriose monomer | glucose |
| maltose monomer | glucose |
| trehalose monomer | glucose |
| lactose monomer | galactose, glucose |
| sucrose monomer | fructose, glucose |
| cellulose monomer | glucose |
| glycogen monomer | glucose |
| amylopectin monomer | glucose |
| amylose monomer | glucose |
| amylose digestive enzyme and breakdown products | salivary & oancreatic a-amylase ~ maltose, maltotriose, oligosaccharides |
| amylopectin digestive enzyme and breakdown products | salivary & pancreatic a-amylase ~ maltose, maltotriose, a-dextrin, oligosaccharides |
| glycogen digestive enzyme and breakdown products | maltose, maltotriose, a-dextrin, oligosaccharides |
| cellulose digestive enzyme and breakdown products | indigestible in humans |
| suctrose digestive enzyme and breakdown products | sucrase-isomaltase ~ glucose, fructose |
| lactose digestive enzyme and breakdown products | lactase ~ glucose, galactose |
| trehalose digestive enzyme and breakdown products | trehalase ~ glucose, glucose |
| maltose digestive enzyme and breakdown products | glucoamylase & sucrase-isomaltase ~ glucose, glucose |
| maltotriose digestive enzyme and breakdown products | glucoamylase & ssucrase-isomaltase ~ glucose, glucose, glucose |
| a-dextrin digestive enzyme and breakdown products | sucrase-isomaltase ~ glucose |
| raffinose digestive enzyme and breakdown products | a-galactosidase (not in humans) ~ D-galactose, sucrose |
| cellobiose digestive enzyme and breakdown products | not digestible in humans |
| site of absorption in monosaccharides | lumen of small intestine |
| how glucose and galactose are transported into cell | via Na+ - Dependent Monosaccharide Cotransport System (Active transport) |
| fructose transporter | GLUT-5 |
| transporter for all 3 monosaccharides - glucose, galactose, fructose | GLUT-2 |
| TRANSPORTER - RBCs and blood-brain barrier | GLUT-1 |
| TRANSPORTER - liver, pancreatic B-cells, contraluminal membrane of intestinal epithelial cells | GLUT-2 |
| TRANSPORTER - brain (neurons) | GLUT-3 |
| TRANSPORTER - adipose tissue,skeletal muscle, heart muscle | GLUT-4 |
| TRANSPORTER - intestinal epithelial cells, sperm | GLUT-5 |
| Na+ - dependent TRANSPORTER - intestinal epithelial cells | SGLT-1 |
| Na+ - dependent TRANSPORTER - kidney (tubular epithelial cells) | SGLT-2 |
| the effect of 50 g of carbs in a particular food on blood glucose levels compared to 50 g of glucose | glycemic index |
| glycemic index times the amount of carbohydrate in standard serving size of that food | glycemic load |
| products of lactase defficiency | short chain fatty acids, lactic acid, gas |
| GLYCOGEN STORAGE DISEASE where G6P is defective and cause enlargement of liver and severe hypoglycemia | #1 Von Gierke's Disease |
| GLYCOGEN STORAGE DISEASE where a-1,4 glucosidase(lysosomal) is defective and causes cardiorespiratory failure which causes death(usually before age 2) | #2 Pompe's Disease |
| GLYCOGEN STORAGE DISEASE where Amylo-1,6-glucosidase is defective and have enlargement of liver(milder) and severe hypoglycemia(milder) but can still make glucose | #3 Cori's Disease |
| GLYCOGEN STORAGE DISEASE where branching enzyme is defective and progressive cirrhosis of the liver - liver damage | #4 Anderson's Disease |
| GLYCOGEN STORAGE DISEASE where phosphorylase is defective and limited ability to perform exercise - painful cramps | #5 McArdle's Disease |
| GLYCOGEN STORAGE DISEASE where phosphorylase enzyme defective enlargement of liver(milder) and hyPERglycemia | #6 Hers' Disease |
Created by:
dkrotin