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1.4

QuestionAnswer
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