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Debranching enzyme has two activities:
One transfers trisaccharide groups
Another one cleaves the remaining single glucose units from the main chain
Glycogeneolysis
Breakdown of glycogen to glucose or glucose 6-phosphate
Glycogenesis
Synthesis of glycogen
Where are glycogenolysis and glycogenesis most important?
Muscle and liver
Gluconeogenesis
Formation of glucose from nonhexose precursors
Where does Gluconeogenesis occur?
All animals, plants, fungi, and microorganisms
In a well fed state, the glucose is provided by...
Dietary carbohydrate
In the well-fed state, what accumulates in the liver?
Glycogen
Once exogenous glucose is exhausted, what maintains blood glucose levels?
Hepatic glycogenolysis
The store of liver glycogen lasts how long during fasting?
12-24 hours
What allows blood glucose to be maintained long after all dietary glucose has been absorbed and completely oxidized?
Gluconeogenesis
Glycogen granules are abundant where during a well fed state? Are they seen in 24-hour fasted animals?
Abundant in the liver in well fed animal, virtually absent in 24-hr fasted animals
During heavy exercise, are glycogen granules present in muscle?
No
In a well-fed human, liver glycogen content can account for how much of the wet weight of the organ?
10%
The muscle stores _______ glycogen than the liver when glycogen is expressed on the same basis. Muscle stores _____% of its wet weight from glycogen
Less, 1-2%
Does the average person have more muscle or more liver? Is there more muscle glycogen or more liver glycogen in the average person?
You have more muscle
There is about twice as much total muscle glycogen as liver glycogen
Glycogen is composed of ________ residues, which are linked in what way?
Glucosyl residues linked by a-1,4-glycosidic linkages
Branches in glycogen are attached through what linkages?
A-1,6-glycosidic linkages
Glycogen's branched structure composed of only one real beginning (reducing end) and many branches terminating with no reducing glucosyl units gives numerous sites of attack for _____________. The multiple sites also function as ________ for the addition of __________ by ___________
Glycogen phosphorylase, primers, glucosyl units, glycogen synthase
To initiate glycogen synthesis, _________ is converted to _________ by _____________
Glucose 6-phosphate, glucose 1-phosphate, phosphoglucomutase
Does phosphoglucomutase function in only glycogen synthesis?
NO
It catalyzes a near equilibrium reaction under intracellular conditions, so it can function in both glycogen degradation and synthesis
What must be present for this reaction to occur?
A bisphosphate compound as an intermediate
Glucose 1,6-bisphosphate
Glucose 6-phosphate to glucose 1,6-bisphosphate is catalyzed by
Phosphoglucokinase
UDP-glucose is called...
Activated glucose - it has a large negative free energy of hydrolysis, used to build the glycogen molecule
What is UDP-glucose formed by?
Glucose 1-phosphate uridylyltransferase
Formation of UDP glucose is...
Energetically favorable and irreversible
What does glycogen synthase do?
Transfers the activated glucosyl moiety of UDP-glucose to a glycogen molecule to form a new glycosidic bond between C1 of the activated sugar and C4 of the glucosyl residue of the growing chain
The reducing end of glucose is always added to what end of the glycogen chain?
Nonreducing
UDP formed as a product of glycogen synthase is converted back to _______ by ____________
UTP, nucleoside diphosphate kinase
__________ functions as a primer for glycogen synthesis
Glycogenin
Glycogenin is what type of enzyme?
Self-glucosylating
Glycogenin uses ________ to link glucose to one of its own __________ residues
UDP-glucose, tyrosine
Where is Glycogenin found in glycogen?
Buried within the molecule, covalently attached to the single reducing end of the glycogen molecule
Does glycogen synthase create the chains of a1-4 glycosidic linkages in glycogen molecules or the a1-6 glycosidic branches found in glycogen?
Only the a1-4 chains, not the branches
What enzyme catalyzes the branching of glycogen?
1-4 a-glucan branching enzyme
How does (1-4)a-Glucan branching enzyme work?
It removes a block of about 7 glucosyl residues from a growing chain in glycogen and transfers it to another chain to produce a1-6 linkage
The creation of a highly branched structure of glycogen requires the concerted efforts of...
Glycogen synthase and branching enzyme
What does glycogen phosphorylase do?
Causes phosphorolysis of glycogen
Pi is used in the cleavage of an a1-4 glycosidic linkage to yield glucose 1-phosphate
Where does phosphorolysis of glycogen occur?
Nonreducing end of glycogen molecule
Amylase
Degrades glycogen and starch in the gut
How does a-amylase work?
Simple hydrolysis
How does the way a-amylase works differ from glycogen phosphorylase?
A-amylase uses simple hydrolysis, using water
Glycogen phosphorylase uses an inorganic phosphate to cleave a1-4 glycosidic bonds
Glycogen phosphorylase stops attacking a1-4 glycosidic linkages how many residues from an a1-6 branch point?
4
A glycogen molecule that has been degraded by phosphorylase to the limit caused by the branches is called...
Phosphorylase-limit dextrin
What is needed to continue degrading glycogen with glycogen phosphorylase?
Debranching enzyme
What does the debranching enzyme do?
1. cleaves 3/4 glucosyl residues of a branch and transfers them to the end of a glucosyl residue on the glycogen chain
2. hydrolyzes the remaining single glucosyl residue
Glycogen serves as a fuel reserve for the synthesis of ________ within ________
ATP, muscle
Liver glycogen functions as a ___________ reserve
Glucose
When are liver glycogen levels high and low?
High- after intaking food
Decreases slowly as it is mobilized to help maintain a nearly constant blood glucose level
Glycogen synthase and glycogen phosphorylase are ________ regulated
Reciprocally
An increase in cAMP signals activation of _____________ and an inactivation of _____________
Glycogen phosphorylase, glycogen synthase
Glycogen phosphorylase occurs in two forms:
a: catalytically active form
b: catalytically inactive form
How is phosphorylase b converted to a (active)?
phosphorylase kinase
What does adenylate cyclase cause formation of?
cAMP
cAMP activates
PKA
PKA turns on
phosphorylase kinase
Phosphorylase kinase
Converts inactive phosphorylase to active phosphorylase
Phosphoprotein phosphatase 1 (PP1)
removes phosphoryl groups from phosphorylase a, converting it to the less active form, phosphorylase b