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32 Cards in this Set

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irreversible enzymes in gluconeogenesis (4)
glucose 6-phosphatase (glucose 6-phosphate --> glucose)

fructose 1,6-bisphosphatase (fructose 1,6-bisphosphate --> fructose 6-phosphate)

phosphoenolpyruvate carboxykinase [PEPCK] (oxaloacetate -->
phosphoenolpyruvate [PEP])

pyruvate carboxylase
(pyruvate --> oxaloacetate)
pyruvate carboxylase:

cofactor

activated by
biotin

acetyl-CoA (from β-oxidation)
step in gluconeogensis that requires ATP
pyruvate --> oxaloacetate
(via pyruvate carboxylase)
step in gluconeogenesis that requires GTP
oxaloacetate --> phosphoenolpyruvate [PEP]
(via PEPCK)
organs/cells that particpate in gluconeogenesis (3)
liver
kidney
intestinal epithelium

(muslce CANNOT participate in gluconeogenesis)
oxaloacetate leaves the mitochondira as
malate (malate shuttle)
fructose 1,6-bisphosphatase:

activated by (1)

inhibited by (2)
ATP

AMP
fructose 2,6-bisphosphate (produced by PFK-2)
enzymes involved in glycogen synthesis
glycogen synthase
branching enzyme
function of glycogen synthase
forms α1,4 glycosidic bonds of linear glucose
function of branching enzyme
hydrolyzes α1,4 glycosidic bond and forms α1,6 glycosidic bond between hydrolyzed oligoglucose and linear glucose
enzymes involved in glycogenolysis
glycogen phosphorylase (α1,4 glucosidase)
debranching enzyme (α1,6 glucosidase)
function of glycogen phosphorylase
breaks α1,4 glycosidic bonds of linear glucose
functions of debranching enzyme (2)
hydrolyzes α1,4 glycosidic bond nearest a branch point (limit dextran) and transfers the oligoglucose unit to the end of linear glucose

hydrolyzes the α1,6 glycosidic bond to release a single molecule of glucose
glycogen storage diseases (4)
Von Gierke's (type I)
Pompe's (type II)
Cori's (type III)
McArdle's (type V)

Very Poor Carbohydrate Metabolizers
Von Gierke's disease:

deficient enzyme
glucose 6-phosphatase
disease:

severe fasting hypoglycemia
↑↑ glycogen in the liver --> hepatomegaly
↑ lactate
Von Gierke's disease
Pompe's disease:

deficient enzyme
lysosomal α1,4 glucosidase (acid maltase)
Pompe's disease:

sign
cardiomegaly
Cori's disease:

deficient enzyme
debranching enzyme (α1,6 glucosidase)
disease:

fasting hypoglycemia
↑ glycogen in the liver --> hepatomegaly
Cori's disease
McArdle's disease:

deficient enzyme
skeletal muscle glycogen phosphorylase
McArdle's disease:

signs (2)
painful muscle cramps and myoglobinuria with strenuous exercise
enzymes involved in the hexose monophosphate (HMP) shunt (2)
glucose 6-phosphate dehydrogenase (G6PD)
transketolase
glucose 6-phosphate dehydrogenase:

stimulated by (2)

inhibited by
insulin
NADP+

NADPH
products of the HMP shunt (2) and their uses
NAPDH - fatty acid and steroid synthesis(anabolic processes); reduces glutathione (GSSG) inside RBC's

ribose 5-phosphate - nucleotide synthesis
sites of the HMP shunt (3)
lactating mammary glands
liver
adrenal cortex

(sites of fatty acid or steroid synthesis - require NADPH)
transketolase:

cofactor
TPP (thiamine)
microscopic evidence of G6PD deficiency
Heinz bodies (altered Hb that precipitates within RBC's)
G6PD deficiency:

MOI
XLR
Cori cycle
lactate from muscle and RBC's is transported to the liver where it is converted to glucose and sent back to the muscle and RBC's
Cori cycle:

net ATP
2 (anaerobic glycolysis)
Aerobic metabolism:

net ATP
- malate shuttle
- G3P shuttle
malate shuttle - 38 ATP

G3P shuttle - 36 ATP