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6.3 Introduction to Metabolism
Terms in this set (20)
most glycogen storage diseases (↑* glycogen metabolism
1/20,000 live births)
Von Gierke Disease (GSD 1a)
Most common glycogen storage disease
removes phosphate from glucose 6-phosphate
liver, kidney (where glycogen is stored)
where is glucose-6-phosphatase expressed?
Storage form of glucose, synthesized during the "fed" state
blood glucose is low (liver, kidney), during exercise (skeletal muscle)
when is glycogen degraded?
Maintain blood glucose homeostasis,
Rapid energy during muscle contraction
why store and degrade glycogen?
removes a terminal glucose-1-phosphate, removes the phosphate
glycogen degradation (People with GSD I usually lack the enzyme for the second step, glucose-6-phosphatase)
fasted state: Glycogen degradation in liver maintains blood glucose ______
______________ maintains blood glucose during a prolonged fast
fatty acids (fasted state)
Taken up---> acetylCoA
--->released as ketone bodies into blood---> slight acidification
Store as glycogen,
But liver enlargement
What happens when a patient with GSD I consumes a carbohydrate rich meal?
No (have to continually eat small meals instead)
Can gluconeogenesis maintain blood glucose in a patient with GSD Ia during a fast?
lactic acidemia, elevated triacylglycerol
Liver cannot generate glucose and release it.
Liver is making lots of G-6-P through glycogen degradation.
What happens to it?
synthesis of Ribose 5-P and NADPH,
path largely driven by substrate availability,
major product is Ribose 5-P.
functions of the pentose phosphate pathway
excess ribose-5-P from PPP
from chronic hyperuricemia
↓↓intake = (hypoglycemia, hyperlactacidemia and hyperuricemia) retarded growth
↑↑ intake = glycogen overload, hepatomegaly and hyperlipidemia, and causes obesity
hyperalaninemia, poor muscle tone
other symptoms of GSD-1
excessive pyruvate leads to this
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